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Progeria
Classification and external resources
ICD-10 E34.8
ICD-9 259.8
OMIM 176670
DiseasesDB 10704
eMedicine derm/731
MeSH D011371
.Progeria (also known as "Hutchinson–Gilford progeria syndrome"[1] and "Hutchinson–Gilford syndrome"[2]) is an extremely rare, severe, genetic condition wherein symptoms resembling aspects of aging are manifested at an early age.^ Hutchinson-Gilford progeria syndrome .
  • Hutchinson-Gilford progeria syndrome - Genetics Home Reference 17 January 2010 4:58 UTC ghr.nlm.nih.gov [Source type: Academic]

^ The first one is known as rare Hutchinson-Gilford syndrome and it is a very rare occurrence.
  • Progeria Syndrome - History, Causes, Signs, Symptoms, Types, Treatments 17 January 2010 4:58 UTC www.wisedude.com [Source type: FILTERED WITH BAYES]

^ A clinical view of Hutchinson-Gilford progeria .
  • Progeria | LIVESTRONG.COM 17 January 2010 4:58 UTC www.livestrong.com [Source type: FILTERED WITH BAYES]

.The disorder has a very low incidence and occurs in one per eight million live births.^ The disorder has a very low incidence and ...
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC health.kosmix.com [Source type: FILTERED WITH BAYES]
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC www.kosmix.com [Source type: Academic]

^ The disorder has a very low incidence and occurs in one per eight million live births.Those born with progeria live for about thirteen years.
  • Reel or real? .:. newkerala.com Online News -27000 17 January 2010 4:58 UTC www.newkerala.com [Source type: General]

^ The disorder has a very low inciden...
  • progeria 17 January 2010 4:58 UTC www.mahalo.com [Source type: General]

[3] .Those born with progeria typically live about thirteen years, although many have been known to live into their late teens and early twenties and rare individuals may even reach their forties[4][5].^ In some cases, there are some who have lived on into their late teens and early twenties, and some have even reached their forties.
  • What Is Progeria Disease � Hutchinson-Gilford Progeria Syndrome 17 January 2010 4:58 UTC lifestyle.iloveindia.com [Source type: FILTERED WITH BAYES]

^ Children generally live only into their early teen years, with the oldest Progeria child living to 21.
  • The Daily Ping: Progeria 17 January 2010 4:58 UTC www.dailyping.com [Source type: General]

^ Those born with progeria typically live about thirteen years, although many have been known to live into their late teens and early twenties and rare individuals may even reach their forties.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

.It is a genetic condition that occurs as a new mutation and is not usually inherited, although there is a uniquely heritable form.^ What are the different ways in which a genetic condition can be inherited?
  • Hutchinson-Gilford progeria syndrome - Genetics Home Reference 17 January 2010 4:58 UTC ghr.nlm.nih.gov [Source type: Academic]

^ It is a genetic condition that occurs as a new mutation and is not usually inherited, although there is a uniquely inheritable form.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]
  • Reel or real? .:. newkerala.com Online News -27000 17 January 2010 4:58 UTC www.newkerala.com [Source type: General]

^ Sporadic means that the mutation occurs at random, and is not usually inherited from a parent.
  • WikiAnswers - How is progeria inherited 17 January 2010 4:58 UTC wiki.answers.com [Source type: General]

.This is in contrast to another rare but similar premature aging syndrome, dyskeratosis congenita (DKC), which is inheritable and will often be expressed multiple times in a family line.^ There is another similar premature aging syndrome called DKC or Dyskeratosis Congenita, which is inheritable and HGPS should not be confused with this.
  • What Is Progeria Disease � Hutchinson-Gilford Progeria Syndrome 17 January 2010 4:58 UTC lifestyle.iloveindia.com [Source type: FILTERED WITH BAYES]

^ Premature aging syndromes (progeria).
  • Premature aging syndromes (progeria). DermNet NZ 17 January 2010 4:58 UTC www.dermnetnz.org [Source type: Academic]
  • PROGERIA at www.schiffszimmerer.de! 17 January 2010 4:58 UTC www.schiffszimmerer.de [Source type: Academic]

^ This is in contrast to another rare but similar premature aging syndrome, dyskeratosis congenita (DKC), which is inheritable and will often be expressed multiple times in a family line.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

[6]
.Scientists are particularly interested in progeria because it might reveal clues about the normal process of aging.^ Progeria is not exactly the same as normal aging.
  • Progeria treatment breakthrough - ImmInst.org Forums 17 January 2010 4:58 UTC www.imminst.org [Source type: FILTERED WITH BAYES]

^ Scientists are particularly interested in progeria because it might reveal clues about the normal process of aging.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]
  • Hutchinson-Gilford Progeria Syndrome - Zimbio 17 January 2010 4:58 UTC www.zimbio.com [Source type: FILTERED WITH BAYES]
  • Hutchinson-Gilford Progeria Syndrome - Zimbio 17 January 2010 4:58 UTC www.zimbio.com [Source type: FILTERED WITH BAYES]

^ Scientists are very interested in progeria because it might reveal clues about the normal process of aging.progeria were first described by Jonathan Hutchinson and it is also described by Hastings Gifford.
  • HEALTH AND BEAUTY 17 January 2010 4:58 UTC www.ethnicexpo.net [Source type: FILTERED WITH BAYES]

[7][8][9] .Progeria was first described in 1886 by Jonathan Hutchinson[10] and also described independently in 1897 by Hastings Gilford.^ Progeria was first described by Jonathan Hutchison in 1886 and later by Hastings Gilford in 1897, and hence the name.
  • What is Progeria 17 January 2010 4:58 UTC www.ygoy.com [Source type: General]

^ Jonathan Hutchinson and Hastings Gilford discovered the syndrome in 1886.

^ This aging disorder was first identified by English surgeons, Jonathan Hutchinson and Hastings Gilford in 1886.
  • Progeria Accelerate Aging Disease HGPS Alopecia Hair Loss Werner Syndrome Cockayne 17 January 2010 4:58 UTC www.encyclocenter.com [Source type: Academic]

[11] .The condition was later named Hutchinson-Gilford Progeria syndrome (HGPS).^ DeBusk FL. The Hutchinson-Gilford progeria syndrome.
  • Hutchinson-Gilford Progeria: eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]
  • Hutchinson-Gilford Progeria: Multimedia - eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

^ Hutchinson-Gilford progeria syndrome (HGPS, progeria) is defined by the presence of the LMNA G608G mutation .
  • Hutchinson-Gilford Progeria Syndrome -- GeneReviews -- NCBI Bookshelf 17 January 2010 4:58 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ A clinical view of Hutchinson-Gilford progeria .
  • Progeria | LIVESTRONG.COM 17 January 2010 4:58 UTC www.livestrong.com [Source type: FILTERED WITH BAYES]

Contents

Symptoms

.The earliest symptoms include failure to thrive and a localized scleroderma-like skin condition.^ Symptoms The earliest symptoms include failure to thrive (FTT) and a localized scleroderma-like skin condition.
  • Hutchinson-Gilford Progeria Syndrome - Zimbio 17 January 2010 4:58 UTC www.zimbio.com [Source type: FILTERED WITH BAYES]
  • Hutchinson-Gilford Progeria Syndrome - Zimbio 17 January 2010 4:58 UTC www.zimbio.com [Source type: FILTERED WITH BAYES]

^ The skin changes showing symptoms of scleroderma.

^ The earliest symptoms include failure to thrive and a localized scleroderma-like skin condition.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

.As a child ages past infancy, additional conditions become apparent.^ As a child ages past infancy, additional conditions become apparent.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Because of this accelerated aging, a child of ten years will have similar respiratory, cardiovascular, and arthritic conditions that a 70-year-old would have.

^ If Aamir Khan's movie Taare Zameen Par focused on dyslexia, Paa focuses on the extremely rare condition called progeria, where a child ages ...

.Limited growth, alopecia, and a distinctive appearance (small face and jaw, pinched nose) are all characteristic of progeria.^ Limited growth, alopecia, and a distinctive appearance (small face and jaw, pinched nose) are all characteristic of progeria.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ As he grows, however, the recognizable traits of Progeria will begin to show up: limited growth resulting in a short stature, hair loss, a small face, thin skin and a loss of body fat.
  • Aging Too Fast: Ky. Boy Faces Progeria - ABC News 17 January 2010 4:58 UTC abcnews.go.com [Source type: General]
  • Baby's rare disease accelerates aging - Kids and parenting- msnbc.com 17 January 2010 4:58 UTC www.msnbc.msn.com [Source type: News]

^ While possessing normal intelligence, affected children develop a distinctive appearance characterized by baldness, aged-looking skin, a pinched nose, and a small face and jaw relative to head size.
  • Genome.gov | Learning About Progeria 17 January 2010 4:58 UTC www.genome.gov [Source type: Academic]
  • Progeria Information 17 January 2010 4:58 UTC www.progeriaeng.centerall.com [Source type: FILTERED WITH BAYES]
  • Progeria 17 January 2010 4:58 UTC www.docstoc.com [Source type: FILTERED WITH BAYES]

.People diagnosed with this disorder usually have small, fragile bodies, like those of elderly people.^ People diagnosed with this disorder usually have small, fragile bodies, like those of elderly people.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Small fragile bodies like those of elderly people.
  • What Is Progeria Disease � Hutchinson-Gilford Progeria Syndrome 17 January 2010 4:58 UTC lifestyle.iloveindia.com [Source type: FILTERED WITH BAYES]

^ Cataract disorders and Retinal disorders are also common to those with WRN. What is unusual is that people with this disorder isn’t affected by Alzheimer’s diseases or hypertension, which are two of the most common diseases in old people.
  • Essay Depot - Progeria 17 January 2010 4:58 UTC www.essaydepot.com [Source type: FILTERED WITH BAYES]

.Later, the condition causes wrinkled skin, atherosclerosis, and cardiovascular problems.^ Later, the condition causes wrinkled skin, atherosclerosis, and cardiovascular problems.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Some children with progeria undergo coronary artery bypass surgery and/or angioplasty in attempts to ease the life-threatening cardiovascular complications caused by progressive atherosclerosis.
  • Progeria 17 January 2010 4:58 UTC www.docstoc.com [Source type: FILTERED WITH BAYES]

^ It is an extremely rare gene mutation which causes tight skin, poor weight gain, stiff joints, and other conditions commonly associated with aging.
  • Stevensville child growing old at a young age 17 January 2010 4:58 UTC www.wndu.com [Source type: News]

Cause

.Hutchinson-Gilford progeria syndrome (HGPS) is a childhood disorder caused by a point mutation in position 1824 of the LMNA gene, replacing cytosine with thymine, creating an unusable form of the protein Lamin A.^ DeBusk FL. The Hutchinson-Gilford progeria syndrome.
  • Hutchinson-Gilford Progeria: Multimedia - eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

^ Hutchinson-Gilford progeria syndrome .
  • Hutchinson-Gilford progeria syndrome - Genetics Home Reference 17 January 2010 4:58 UTC ghr.nlm.nih.gov [Source type: Academic]

^ LMNA G608G point mutation .
  • Hutchinson-Gilford Progeria Syndrome -- GeneReviews -- NCBI Bookshelf 17 January 2010 4:58 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.Lamin A is part of the building blocks of the nuclear envelope.^ Lamin A plays an important role in building the nuclear envelope, thus, when a mutation occurs in this gene, a mutant form of Lamin A protein is produced which destabilizes the person's cells in the body, thereby conducing to the condition called progeria.
  • Progeria Symptoms 17 January 2010 4:58 UTC www.buzzle.com [Source type: FILTERED WITH BAYES]

^ Causes:- Hutchinson-Gilford progeria syndrome (HGPS) is a childhood disorder caused by a point mutation in position 1824 of the LMNA gene, replacing cytosine with thymine, creating an unusable form of the protein Lamin A. Lamin A is part of the building blocks of the nuclear envelope.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Lamin A cannot be produced, and prelamin A builds up on the nuclear membrane, causing a characteristic nuclear blebbing.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

.Unlike most other "accelerated aging diseases" (such as Werner's syndrome, Cockayne's syndrome, or xeroderma pigmentosum), progeria is not caused by defective DNA repair.^ Examples Some of the examples include: Ataxia telangiectasia Bloom syndrome Cockayne’s syndrome Progeria (Hutchinson-Gilford Progeria syndrome) Rothmund-Thomson syndrome Trichothiodystrophy Werner syndrome Xeroderma pigmentosum DNA repair defects distinguished from “accelerated aging” Most of the DNA repair deficiency diseases ...
  • Hutchinson-Gilford Progeria Syndrome - Zimbio 17 January 2010 4:58 UTC www.zimbio.com [Source type: FILTERED WITH BAYES]
  • Hutchinson-Gilford Progeria Syndrome - Zimbio 17 January 2010 4:58 UTC www.zimbio.com [Source type: FILTERED WITH BAYES]

^ Genome instability and DNA repair defects have been discovered in the premature aging disease Hutchinson-Gilford progeria syndrome.

^ Progeria is the most radical of the ageing illnesses.

Because these diseases display different aspects of aging but never every aspect, they are often called "segmental progerias".[citation needed]

Diagnosis

.Diagnosis is suspected according to signs and symptoms, such as skin changes, abnormal growth, and loss of hair.^ Diagnosis:- Diagnosis is suspected according to signs and symptoms, such as skin changes, abnormal growth, and loss of hair.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ By 12 months, signs and symptoms, such as skin changes and hair loss, begin to appear.

^ As he grows, however, the recognizable traits of Progeria will begin to show up: limited growth resulting in a short stature, hair loss, a small face, thin skin and a loss of body fat.
  • Aging Too Fast: Ky. Boy Faces Progeria - ABC News 17 January 2010 4:58 UTC abcnews.go.com [Source type: General]
  • Baby's rare disease accelerates aging - Kids and parenting- msnbc.com 17 January 2010 4:58 UTC www.msnbc.msn.com [Source type: News]

.It can be confirmed through a genetic test.^ It can be confirmed through a genetic test.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Progeria is a rare genetic disorder that is diagnosed through blood tests to look for a specific gene mutation.
  • Questions about Progeria - Ask.com 17 January 2010 4:58 UTC www.ask.com [Source type: FILTERED WITH BAYES]

^ However, it is confirmed only after carrying out a genetic test.
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC health.kosmix.com [Source type: FILTERED WITH BAYES]
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC www.kosmix.com [Source type: Academic]
  • What is Progeria 17 January 2010 4:58 UTC www.ygoy.com [Source type: General]

[12]

Treatment

.No treatments have been proven effective.^ Treatment:- No treatments have been proven effective.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ There are right now no effective treatments for Progeria.
  • What Is Progeria Disease � Hutchinson-Gilford Progeria Syndrome 17 January 2010 4:58 UTC lifestyle.iloveindia.com [Source type: FILTERED WITH BAYES]

^ There is no single treatment, and the effectiveness of particular interventions is supported by only limited data.
  • Reel or real? .:. newkerala.com Online News -27000 17 January 2010 4:58 UTC www.newkerala.com [Source type: General]

.Most treatment focuses on reducing complications (such as cardiovascular disease) with heart bypass surgery or low-dose aspirin.^ Most treatment focuses on reducing complications (such as cardiovascular disease) with heart bypass surgery or low-dose aspirin.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Treatments can only reduce complications like cardiovascular diseases, with a possible heart bypass operation or low-dose medicines.
  • Progeria symptoms and Progeria treatment | KRARAN 17 January 2010 4:58 UTC kraran.com [Source type: General]

^ To reduce these risks a low daily dose aspirin is prescribed.

[13] .Children may also benefit from a high-calorie diet.^ Children may also benefit from a high-calorie diet.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Including extra calories in your child's daily diet may help prevent weight loss and ensure adequate nutrition.
  • Progeria 17 January 2010 4:58 UTC www.cnn.com [Source type: General]
  • Progeria: Treatments and drugs - MayoClinic.com 17 January 2010 4:58 UTC www.mayoclinic.com [Source type: FILTERED WITH BAYES]
  • Progeria 17 January 2010 4:58 UTC edition.cnn.com [Source type: General]

^ They use high-calorie diets to prevent weight loss and physical therapy but why some people in the forum use calorie restriction to prevent aging ?
  • Progeria treatment breakthrough - ImmInst.org Forums 17 January 2010 4:58 UTC www.imminst.org [Source type: FILTERED WITH BAYES]

.Growth hormone treatment has been attempted.^ Growth hormone treatment has been attempted.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ There is a growth hormone treatment being tested to find out if it can cure progeria.
  • Progeria symptoms and Progeria treatment | KRARAN 17 January 2010 4:58 UTC kraran.com [Source type: General]

^ Investigational Therapies Since some studies suggest that some symptoms of Hutchinson-Gilford Progeria may be due to the production of a biologically inactive form of growth hormone and treatment of affected children with recombinant human growth hormone (hGH) has been attempted.
  • The Progeria Project Foundation - Progeria? - What is Progeria? 17 January 2010 4:58 UTC www.progeriaproject.com [Source type: Academic]

[14]
.A type of anticancer drug, the farnesyltransferase inhibitors (FTIs), has been proposed, but their use has been mostly limited to animal models.^ A type of anticancer drug, the farnesyltransferase inhibitors (FTIs), has been proposed, but their use has been mostly limited to animal models.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Drugs known as farnesyltransferase inhibitors (FTIs), which were developed for treating cancer, have shown promise in laboratory studies in correcting the cell defects that cause progeria.

^ Life Extension Daily reported an study out of UCLA that in mouse studies using an experimental cancer drug ''Farnesyltransferase inhibitor'' (FTI) that slowed Progeria in these animals.
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC health.kosmix.com [Source type: FILTERED WITH BAYES]
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC www.kosmix.com [Source type: Academic]

[15] A Phase II clinical trial using the FTI Lonafarnib began in May 2007.[16]

Prognosis

.There is no known cure.^ There is no known prevention.
  • Progeria Medical Information 17 January 2010 4:58 UTC www.drugs.com [Source type: Academic]
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC health.kosmix.com [Source type: FILTERED WITH BAYES]
  • Progeria - Symptoms, Diagnosis, Treatment of Progeria - NY Times Health Information 17 January 2010 4:58 UTC health.nytimes.com [Source type: News]
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC www.kosmix.com [Source type: Academic]
  • Progeria 17 January 2010 4:58 UTC premierhealthpartners.adam.com [Source type: FILTERED WITH BAYES]

^ To date, there is no known cure for progeria.

^ Prevention: There is no known prevention.
  • Progeria: Progeria is a disease that produces rapid aging, beginning ... 17 January 2010 4:58 UTC www.henryfordhealth.org [Source type: Academic]

.Few people with progeria exceed 13 years of age.^ Few people with progeria exceed 13 years of age.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Children with progeria die of heart disease (atherosclerosis) at an average age of 13 years, with a range of about eight to 21 years.
  • Progeria, Hutchinson Gilford 17 January 2010 4:58 UTC children.webmd.com [Source type: General]
  • Evansville,IN Progeria, Hutchinson Gilford; HGPS Hutchinson-Gilford ... 17 January 2010 4:58 UTC www.stmarys.org [Source type: FILTERED WITH BAYES]

^ In Paa, Amitabh Bachchan plays the role of 13 year old affected by Progeria.
  • Thanks to Paa, Bajaj Allianz brings Progeria awareness - Bollywood Movie News - IndiaGlitz.com 17 January 2010 4:58 UTC www.indiaglitz.com [Source type: FILTERED WITH BAYES]

[17] .At least 90% of patients die from complications of atherosclerosis, such as heart attack or stroke.^ At least 90% of patients die from complications of atherosclerosis, such as heart attack or stroke.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Heart attack ( myocardial infarction) Stroke .
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC health.kosmix.com [Source type: FILTERED WITH BAYES]
  • Progeria - Symptoms, Diagnosis, Treatment of Progeria - NY Times Health Information 17 January 2010 4:58 UTC health.nytimes.com [Source type: News]
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC www.kosmix.com [Source type: Academic]

^ Review medical complications possibly associated with Progeria: Atherosclerosis during childhood Coronary disease ( see Heart disease ) Premature death usually by 27 years Stroke more complications...» Research More About Progeria .
  • Symptoms of Progeria - WrongDiagnosis.com 17 January 2010 4:58 UTC www.wrongdiagnosis.com [Source type: General]

[18]
.Mental development is not affected.^ Mental development is not affected.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Though the problem of Progeria affects the growth and development of a child, it does not lead to mental retardation .
  • Progeria Disease - Mental Retardation - Genetic Mutation - Genetic Disorder » Causes and Symptoms of Progeria 17 January 2010 4:58 UTC www.ayushveda.com [Source type: General]

.The development of symptoms is comparable to aging at a rate eight to ten times faster than normal, although certain age-related conditions do not occur.^ It is when you age much faster then normal.
  • The My Hero Project - John Tacket 17 January 2010 4:58 UTC www.myhero.com [Source type: General]

^ Progeria symptoms comprise normal aging signs occurring in children.

^ The development of symptoms is comparable to aging at a rate eight to ten times faster than normal, although certain age-related conditions do not occur.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

.Specifically, patients show no neurodegeneration or cancer predisposition.^ Specifically, patients show no neurodegeneration or cancer predisposition.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ No marked radiosensitivity was found among strains derived from patients with a number of other conditions associated with a predisposition to malignancy.
  • Progeria :: physiopathology 17 January 2010 4:58 UTC lib.bioinfo.pl [Source type: Academic]

.They do not develop physically mediated "wear and tear" conditions commonly associated with aging, like cataracts (caused by UV exposure) and osteoarthritis (caused by mechanical wear).^ They do not develop physically mediated “wear and tear” conditions commonly associated with aging, like cataracts (caused by UV exposure) and osteoarthritis (caused by mechanical wear).
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ The development of symptoms is comparable to aging at a rate eight to ten times faster than normal, although certain age-related conditions do not occur.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ They do not deal with madness in any form but some kind of mental deficiency per se or some physical trigger, like an accident that could lead to a mental deficiency.
  • Reel or real? .:. newkerala.com Online News -27000 17 January 2010 4:58 UTC www.newkerala.com [Source type: General]

[12]
Although there may not be any successful treatments for Progeria itself, there are treatments for the problems it causes, such as arthritic, respiratory, and cardiovascular problems

Epidemiology

.A study from the Netherlands has shown an incidence of 1 in 4 million births.^ Epidemiology:- A study from the Netherlands has shown an incidence of 1 in 4 million births.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ The disorder has a very low incidence and occurs in one per eight million live births.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

[19] .Currently, there are between 35 and 45 known cases in the world.^ Currently, there are between 35 and 45 known cases in the world.Approximately 100 cases have been formally identified in medical history.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ There are currently between 30 and 40 known cases worldwide of Progeria.
  • progeria@Everything2.com 17 January 2010 4:58 UTC www.everything2.com [Source type: FILTERED WITH BAYES]

^ There are currently between 35 and 45 known cases worldwide of Progeria.

[20] .Approximately 100 cases have been formally identified in medical history.^ Currently, there are between 35 and 45 known cases in the world.Approximately 100 cases have been formally identified in medical history.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Approximately 100 cases have been formally identified in medical history and the odds of being diagnosed with it are roughly one in 8 million.
  • Baby's rare disease accelerates aging - Kids and parenting- msnbc.com 17 January 2010 4:58 UTC www.msnbc.msn.com [Source type: News]

^ Approximately 100 cases of HGPS have been reported in the literature.
  • Hutchinson-Gilford Progeria: eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

[17][21]
.Classical Hutchinson-Gilford Progeria Syndrome is almost never passed on from parent to child.^ DeBusk FL. The Hutchinson-Gilford progeria syndrome.
  • Hutchinson-Gilford Progeria: eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]
  • Hutchinson-Gilford Progeria: Multimedia - eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

^ Classical Hutchinson-Gilford Progeria Syndrome is almost never passed on from parent to child.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Alternative Names Hutchinson-Gilford syndrome .

.It is usually caused by a new (sporadic) mutation during the early division of the cells in the child.^ It is usually caused by a new (sporadic) mutation during the early division of the cells in the child.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Ninety percent of children with progeria have a mutation on the gene that encodes the protein lamin A. Progeria usually occurs sporadically, and is not seen in siblings of affected children, although -- in very rare circumstances -- more than one child in a family can have progeria.

^ However, Progeria is an autosomal dominant ageing disease which is caused by an sporadic mutation in the LMNA-gene.
  • Progeria.be - Information 17 January 2010 4:58 UTC www.progeria.be [Source type: FILTERED WITH BAYES]

.It is usually genetically dominant; therefore, parents who are healthy will normally not pass it on to their children.^ It is usually genetically dominant; therefore, parents who are healthy will normally not pass it on to their children.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Children with progeria usually appear normal at birth and in early infancy.
  • Hutchinson-Gilford Progeria Syndrome -- GeneReviews -- NCBI Bookshelf 17 January 2010 4:58 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ As newborns, children with Progeria usually appear normal.

[8] .Affected children rarely live long enough to have children themselves.^ Affected children rarely live long enough to have children themselves.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Progeria is a rare medical disorder which affects children ...

^ Progeria, which affects an estimated one in four million to one in eight million live births, is the result of a mutation in a gene that causes the children to make an abnormal form of protein.
  • Dearth of patients makes progeria trial uncertain 17 January 2010 4:58 UTC www.post-gazette.com [Source type: FILTERED WITH BAYES]

.There have been only two known cases in which it became evident that a healthy parent can carry the LMNA mutation that causes progeria.^ There is only 17 individuals alive today known to have Progeria.
  • informative speech 17 January 2010 4:58 UTC www.uni.edu [Source type: FILTERED WITH BAYES]

^ The mutations to the gene LMNA, which encodes for the Lamin-A protein, causes Progeria.
  • Essay Depot - Progeria 17 January 2010 4:58 UTC www.essaydepot.com [Source type: FILTERED WITH BAYES]

^ There have been only two known cases in which it became evident that a healthy parent can carry the LMNA mutation that causes progeria.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

.A family from India has five children with progeria;[22] they were the subject of a 2005 Bodyshock documentary entitled The 80 Year Old Children.^ A family from India has five children with progeria; they were the subject of a 2005 Bodyshock documentary entitled The 80 Year Old Children.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ WHAT ARE THE FEATURES OF PROGERIA? Although they are born looking healthy, children with Progeria begin to display many characteristics of accelerated aging at around 18-24 months of age.
  • Progeria - This Child will Break your Heart, page 1 17 January 2010 4:58 UTC www.abovetopsecret.com [Source type: General]

^ Children with progeria die of heart disease (atherosclerosis) at an average age of 13 years, with a range of about eight to 21 years.
  • Progeria, Hutchinson Gilford 17 January 2010 4:58 UTC children.webmd.com [Source type: General]
  • Evansville,IN Progeria, Hutchinson Gilford; HGPS Hutchinson-Gilford ... 17 January 2010 4:58 UTC www.stmarys.org [Source type: FILTERED WITH BAYES]

.In the other case, a family from Belgium has two children with progeria.^ In the other case, a family from Belgium has two children with progeria.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Contact with other families with Progeria kids : .
  • Progeria.be - Information 17 January 2010 4:58 UTC www.progeria.be [Source type: FILTERED WITH BAYES]

^ A family from India has five children with progeria; they were the subject of a 2005 Bodyshock documentary entitled The 80 Year Old Children.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

[23]

Research

.Several discoveries have been made that have led to greater understanding and perhaps eventual treatment.^ Research:- Several discoveries have been made that have led to greater understanding and perhaps eventual treatment.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

[24]
.A 2003 report in Nature[25] said that progeria may be a de novo dominant trait.^ A 2003 report in Nature said that progeria may be a de novo dominant trait.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ FROM: anthony medford DATE: Tuesday February 18, 2003 -- 4:45:15 pm i am doing a reported on progeria and i am wondering if i can get some pictures.
  • The Daily Ping: Progeria 17 January 2010 4:58 UTC www.dailyping.com [Source type: General]

^ Thus, the recurrence risk may be on the order of one in 500, as in other de novo dominant mutations .
  • Hutchinson-Gilford Progeria Syndrome -- GeneReviews -- NCBI Bookshelf 17 January 2010 4:58 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.It develops during cell division in a newly conceived zygote or in the gametes of one of the parents.^ It develops during cell division in a newly conceived zygote or in the gametes of one of the parents.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Although cells normally multiply during growth and development, the misshapen nucleus cannot divide properly, ultimately damaging cells and accelerating the aging process.
  • Progeria treatment breakthrough - ImmInst.org Forums 17 January 2010 4:58 UTC www.imminst.org [Source type: FILTERED WITH BAYES]

^ This can be happened during the formation of that one sex cell from which the child has arisen, but also earlier (in the parent) or later (in the child).
  • Progeria.be - Information 17 January 2010 4:58 UTC www.progeria.be [Source type: FILTERED WITH BAYES]

.It is caused by mutations in the LMNA (lamin A protein) gene on chromosome 1; the mutated form of lamin A is commonly known as progerin.^ Lamins A and C are formed by alternative splicing of the LMNA gene transcript.
  • Hutchinson-Gilford Progeria Syndrome -- GeneReviews -- NCBI Bookshelf 17 January 2010 4:58 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ HGPS is caused by a mutation in the gene called LMNA (pronounced, lamin a).
  • Progeria.be - Information 17 January 2010 4:58 UTC www.progeria.be [Source type: FILTERED WITH BAYES]

^ It is caused by mutations in the LMNA (lamin A protein) gene on chromosome 1; the mutated form of lamin A is commonly known as progerin .
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

.One of the authors, Leslie Gordon, was a physician who didn't know anything about progeria until her own son, Sam, was diagnosed at 21 months.^ One of the authors, Leslie Gordon, was a physician who didn’t know anything about progeria until her own son, Sam, was diagnosed at 21 months.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ The disorder has a very low incidence and occurs in one per eight million live births.Those born with progeria live for about thirteen years.
  • Reel or real? .:. newkerala.com Online News -27000 17 January 2010 4:58 UTC www.newkerala.com [Source type: General]

^ Amitabh played Abhishek's son who suffers from progeria, a disease that accelerates the ageing process.

.Gordon and her husband, pediatrician Scott Berns, founded the Progeria Research Foundation.^ Gordon and her husband, pediatrician Scott Berns, founded the Progeria Research Foundation.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ In 1999, Dr. Gordon, her husband and her sister, Audrey Gordon, started the Progeria Research Foundation.
  • Dearth of patients makes progeria trial uncertain 17 January 2010 4:58 UTC www.post-gazette.com [Source type: FILTERED WITH BAYES]

^ This information is provided courtesy of The Progeria Research Foundation.

[26]

Lamin A

.Nuclear lamin A is a protein scaffold on the inner edge of the nucleus that helps organize nuclear processes such as RNA and DNA synthesis.^ Lamin A:- Nuclear lamin A is a protein scaffold on the inner edge of the nucleus that helps organize nuclear processes such as RNA and DNA synthesis.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ The nuclear lamins may also play a role in regulating gene expression, DNA synthesis, and DNA repair.
  • Hutchinson-Gilford Progeria: eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

^ The abnormal progerin protein acts in a dominant-negative manner to prevent the normal assembly of nuclear lamins into the nuclear lamina.
  • Hutchinson-Gilford Progeria: eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

.Prelamin A contains a CAAX box at the C-terminus of the protein (where C is a cysteine and A is any aliphatic amino acids).^ Prelamin A contains a CAAX box at the C-terminus of the protein (where C is a cysteine and A is any aliphatic amino acids).
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Because prelamin A contains a CAAX (cysteine / aliphatic / aliphatic / any amino acid) box at its carboxyl terminus, it is modified by farnesylation, whereas lamin C is not.
  • Hutchinson-Gilford Progeria Syndrome -- GeneReviews -- NCBI Bookshelf 17 January 2010 4:58 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ The gene mutation implicated in Progeria causes a section of 50 amino acids within the lamin A protein to be deleted, resulting in a mutated protein that is called progerin.

.This ensures that the cysteine is farnesylated and allows prelamin A to bind membranes, specifically the nuclear membrane.^ This ensures that the cysteine is farnesylated and allows prelamin A to bind membranes, specifically the nuclear membrane.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ After prelamin A has been localized to the cell nuclear membrane, the C-terminal amino acids, including the farnesylated cysteine, are cleaved off by a specific protease.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ The farnesylated preprogerin protein is then incorporated into the nuclear membrane.
  • Hutchinson-Gilford Progeria: eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

.After prelamin A has been localized to the cell nuclear membrane, the C-terminal amino acids, including the farnesylated cysteine, are cleaved off by a specific protease.^ This ensures that the cysteine is farnesylated and allows prelamin A to bind membranes, specifically the nuclear membrane.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ After prelamin A has been localized to the cell nuclear membrane, the C-terminal amino acids, including the farnesylated cysteine, are cleaved off by a specific protease.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Once there, a second tag within the protein signals an enzyme to cleave off the terminal portion of the protein, including the farnesyl group, freeing lamin A to integrate properly into the nuclear lamina.

.The resulting protein, now lamin A, is no longer membrane-bound, and carries out functions inside the nucleus.^ The resulting protein is now lamin A, is no longer membrane-bound, and carries out functions inside the nucleus.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Luckily, lamin A does not appear to be essential; indeed, mouse models in which the genes for prelamin A and C are knocked out show no symptoms.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Lamin A:- Nuclear lamin A is a protein scaffold on the inner edge of the nucleus that helps organize nuclear processes such as RNA and DNA synthesis.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

.In HGPS, the recognition site that the enzyme requires for cleavage of prelamin A to lamin A is mutated.^ In HGPS, the recognition site that the enzyme requires for cleavage of prelamin A to lamin A is mutated.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Cause : HGPS is caused by a mutation in the gene called LMNA (pronounced lamin-a).

^ The G608G mutation and consequent abnormal splicing produces a prelamin A that still retains the CAAX box, but is missing the site for endoproteolytic cleavage.
  • Hutchinson-Gilford Progeria Syndrome -- GeneReviews -- NCBI Bookshelf 17 January 2010 4:58 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.Lamin A cannot be produced, and prelamin A builds up on the nuclear membrane, causing a characteristic nuclear blebbing.^ Lamin A cannot be produced, and prelamin A builds up on the nuclear membrane, causing a characteristic nuclear blebbing.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Hutchinson-Gilford progeria syndrome (HGPS) is caused by a mutation in the lamin A/C gene ( LMNA ); the mutant allele produces a truncated lamin A (”progerin”) that disrupts the nuclear architecture, interferes with cell cycle progression...
  • Progeria | LIVESTRONG.COM 17 January 2010 4:58 UTC www.livestrong.com [Source type: FILTERED WITH BAYES]

^ Both cases result in the buildup of farnesylated prelamin A on the nuclear membrane and in the characteristic nuclear LMNA blebbing.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

[27] .This results in the premature aging symptoms of progeria, although the mechanism connecting the misshapen nucleus to the symptoms is not known.^ Progeria is premature old age.
  • Health and Medicine - What Is Progeria?: Science Fact Finder 17 January 2010 4:58 UTC www.enotes.com [Source type: Academic]

^ This results in the premature aging symptoms of progeria, although the mechanism connecting the misshapen nucleus to the symptoms is not known.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Premature aging syndromes (progeria).

.A study that compared HGPS patient cells with the skin cells from LMNA young and elderly human subjects found similar defects in the HGPS and elderly cells, including down-regulation of certain nuclear proteins, increased DNA damage, and demethylation of histone, leading to reduced heterochromatin.^ Cells from HGPS patients exhibit extensive nuclear defects, including abnormal chromatin structure and increased DNA damage.
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC health.kosmix.com [Source type: FILTERED WITH BAYES]
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC www.kosmix.com [Source type: Academic]

^ A study that compared HGPS patient cells with the skin cells from LMNA young and elderly human subjects found similar defects in the HGPS and elderly cells, including down-regulation of certain nuclear proteins, increased DNA damage, and demethylation of histone, leading to reduced heterochromatin.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Drugs known as farnesyltransferase inhibitors (FTIs), which were developed for treating cancer, have shown promise in laboratory studies in correcting the cell defects that cause progeria.

[28] .Nematodes over their lifespan show progressive lamin changes comparable to HGPS in all cells but neurons and gametes.^ Hutchinson-Gilford progeria syndrome (HGPS) is caused by a mutation in the lamin A/C gene ( LMNA ); the mutant allele produces a truncated lamin A (”progerin”) that disrupts the nuclear architecture, interferes with cell cycle progression...
  • Progeria | LIVESTRONG.COM 17 January 2010 4:58 UTC www.livestrong.com [Source type: FILTERED WITH BAYES]

^ As a result of the absence of lamin A in the nuclear lamina, the cell nuclei from HGPS patients display abnormal nuclear blebbing and aberrant nuclear shapes.
  • Hutchinson-Gilford Progeria: eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

[29] .These studies suggest that lamin A defects contribute to normal aging.^ Many researchers suggest that the normal aging process is due to cumulative cellular damage resulting from ongoing chemical (metabolic) processes within bodily cells.
  • The Progeria Project Foundation - Progeria? - What is Progeria? 17 January 2010 4:58 UTC www.progeriaproject.com [Source type: Academic]

^ Abnormal lamin A (progerin) appears to accumulate with aging in normal cells.
  • NEJM -- Phenotype and Course of Hutchinson-Gilford Progeria Syndrome 17 January 2010 4:58 UTC content.nejm.org [Source type: Academic]

.
A range of putative disease-causing mechanisms for the case of HGPS, in which lamin A is permanently farnesylated in the form of progerin.
^ The premature-ageing disease Hutchinson-Gilford Progeria Syndrome (HGPS) is caused by constitutive production of progerin, a mutant form of the nuclear architectural protein lamin A. Progerin is also expressed sporadically in wild-type cells and has been linked to physiological ageing.
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC health.kosmix.com [Source type: FILTERED WITH BAYES]
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC www.kosmix.com [Source type: Academic]

^ Hutchinson-Gilford progeria syndrome (HGPS) is caused by a mutation in the lamin A/C gene ( LMNA ); the mutant allele produces a truncated lamin A (”progerin”) that disrupts the nuclear architecture, interferes with cell cycle progression...
  • Progeria | LIVESTRONG.COM 17 January 2010 4:58 UTC www.livestrong.com [Source type: FILTERED WITH BAYES]

^ All 3 drugs block the production of the farnesyl molecule that is needed for progerin to create disease in Progeria.
  • Progeria.be - Information 17 January 2010 4:58 UTC www.progeria.be [Source type: FILTERED WITH BAYES]

.We predict that progerin becomes entrapped in the nuclear membrane as a result of permanent farnesylation, resulting in a multitude of downstream effects.^ Because progerin carries the farnesylation tag but lacks the second cleavage tag, Capell speculated that progerin was becoming permanently stuck to the inner nuclear membrane.

^ This ensures that the cysteine is farnesylated and allows prelamin A to bind membranes, specifically the nuclear membrane.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ Farnesylation tethers lamin A to the inner nuclear membrane.

.Disruption of the normal lamina architecture leads to fragility, vulnerability to mechanical stresses and nuclear blebbing.^ The abnormal progerin protein acts in a dominant-negative manner to prevent the normal assembly of nuclear lamins into the nuclear lamina.
  • Hutchinson-Gilford Progeria: eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

^ As a result of the absence of lamin A in the nuclear lamina, the cell nuclei from HGPS patients display abnormal nuclear blebbing and aberrant nuclear shapes.
  • Hutchinson-Gilford Progeria: eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

^ This protein fails to integrate properly into the lamina, thereby disrupting the nuclear scaffolding and causing gross disfigurement of the nucleus.

.Other consequences include disrupted interactions with other nuclear envelope proteins – such as nesprin, emerin and laminaassociated protein 2 (LAP2) – which leads to their mislocalization (that is, emerin is relocalized to the cytoplasm in Lmna -/- mice)74 and clustering of nuclear pores.^ Lamin A:- Nuclear lamin A is a protein scaffold on the inner edge of the nucleus that helps organize nuclear processes such as RNA and DNA synthesis.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ The LMNA genes encodes the nuclear A-type lamins, which are type V intermediate filament proteins that localize to the cell nucleus and form the nuclear lamina, a structure that supports the nuclear envelope.
  • Hutchinson-Gilford Progeria: eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

^ The LMNA gene codes for a protein called lamin A, which constitutes a major component of the scaffold-like network of proteins just inside the cell's nuclear membrane, called the lamina.

.Disorganization and loss of peripheral heterochromatin is also seen, with heterochromatin becoming detached from the nuclear envelope, and disrupted interactions with RNA polymerase II, RNA splicing factors and transcription factors such as the retinoblastoma transcriptional regulator (RB) and sterol response element binding protein (SREBP1), which leads to misregulation of gene expression.^ Lamin A:- Nuclear lamin A is a protein scaffold on the inner edge of the nucleus that helps organize nuclear processes such as RNA and DNA synthesis.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ A study that compared HGPS patient cells with the skin cells from LMNA young and elderly human subjects found similar defects in the HGPS and elderly cells, including down-regulation of certain nuclear proteins, increased DNA damage, and demethylation of histone, leading to reduced heterochromatin.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ The LMNA genes encodes the nuclear A-type lamins, which are type V intermediate filament proteins that localize to the cell nucleus and form the nuclear lamina, a structure that supports the nuclear envelope.
  • Hutchinson-Gilford Progeria: eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

GCL, germ cell-less. Capell and Collins, Nature Reviews Genetics 2006.[30]

Mouse model of progeria

.
Untreated cells from children with the genetic disease progeria (left) compared to similar cells treated with farnesyltransferase inhibitors (FTIs).
^ Progeria is a rare genetic disease that...
  • Progeria | LIVESTRONG.COM 17 January 2010 4:58 UTC www.livestrong.com [Source type: FILTERED WITH BAYES]

^ Progeria children: rapid aging disease .
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC health.kosmix.com [Source type: FILTERED WITH BAYES]

^ Drugs known as farnesyltransferase inhibitors (FTIs), which were developed for treating cancer, have shown promise in laboratory studies in correcting the cell defects that cause progeria.

In the test tube, FTIs reverse the nuclear damage caused by the disease.
.A mouse model of progeria exists, though in the mouse, the LMNA prelamin A is not mutated.^ The diagnosis of Hutchinson-Gilford progeria syndrome (HGPS, progeria) is based on recognition of common clinical features and the presence of the LMNA G608G mutation .
  • Hutchinson-Gilford Progeria Syndrome -- GeneReviews -- NCBI Bookshelf 17 January 2010 4:58 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ However, Progeria is an autosomal dominant ageing disease which is caused by an sporadic mutation in the LMNA-gene.
  • Progeria.be - Information 17 January 2010 4:58 UTC www.progeria.be [Source type: FILTERED WITH BAYES]

^ A transgenic mouse model for HGPS has been created by introducing a splicing defect into intron 9 of the mouse LMNA gene.
  • Hutchinson-Gilford Progeria: eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

Instead, ZMPSTE24, the specific protease that is required to remove the C-terminus of prelamin A, is missing. .Both cases result in the buildup of farnesylated prelamin A on the nuclear membrane and in the characteristic nuclear LMNA blebbing.^ As a result of the absence of lamin A in the nuclear lamina, the cell nuclei from HGPS patients display abnormal nuclear blebbing and aberrant nuclear shapes.
  • Hutchinson-Gilford Progeria: eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

^ Farnesylation tethers lamin A to the inner nuclear membrane.

^ The farnesylated preprogerin protein is then incorporated into the nuclear membrane.
  • Hutchinson-Gilford Progeria: eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

Fong et al. use a .farnesyl transferase inhibitor (FTI) in this mouse model to inhibit protein farnesylation of prelamin A. Treated mice had greater grip strength and lower likelihood of rib fracture and may live longer than untreated mice.^ Drugs known as farnesyltransferase inhibitors (FTIs), which were developed for treating cancer, have shown promise in laboratory studies in correcting the cell defects that cause progeria.

^ So if the FTI drug can block this farnesyl group attachment in children with Progeria, then progerin may be paralyzed and Progeria improved.
  • Progeria.be - Information 17 January 2010 4:58 UTC www.progeria.be [Source type: FILTERED WITH BAYES]

^ Lonafarnib is an FTI (Farnesyltransferase inhibitor), a drug that can reverse an abnormality in Progeria cells in the laboratory, and has improved disease in Progeria mice.
  • Progeria.be - Information 17 January 2010 4:58 UTC www.progeria.be [Source type: FILTERED WITH BAYES]

[31]
.This method does not directly "cure" the underlying cause of progeria.^ Progeria Research Foundation : www.progeriaresearch.org The Progeria Research Foundation raises money and researches Progerias cause as well as possible treatments and cures.

^ The specific underlying cause of the accelerated aging associated with Hutchinson-Gilford Progeria syndrome is not yet known.
  • The Progeria Project Foundation - Progeria? - What is Progeria? 17 January 2010 4:58 UTC www.progeriaproject.com [Source type: Academic]

^ This type of progeria is hereditary and is transmitted as a recessive trait , but its underlying cause is unknown.
  • progeria (pathology) -- Britannica Online Encyclopedia 17 January 2010 4:58 UTC www.britannica.com [Source type: FILTERED WITH BAYES]

.This method prevents prelamin A from going to the nucleus in the first place so that no prelamin A can build up on the nuclear membrane, but equally, there is no production of normal lamin A in the nucleus.^ There is no known prevention.
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC health.kosmix.com [Source type: FILTERED WITH BAYES]
  • Progeria | LIVESTRONG.COM 17 January 2010 4:58 UTC www.livestrong.com [Source type: FILTERED WITH BAYES]

^ The abnormal progerin protein acts in a dominant-negative manner to prevent the normal assembly of nuclear lamins into the nuclear lamina.
  • Hutchinson-Gilford Progeria: eMedicine Dermatology 17 January 2010 4:58 UTC emedicine.medscape.com [Source type: Academic]

^ Farnesylation tethers lamin A to the inner nuclear membrane.

.Luckily, lamin A does not appear to be essential; indeed, mouse models in which the genes for prelamin A and C are knocked out show no symptoms.^ Do you think it is possible to create a set of these based on the procedure for knocking out that target gene?
  • Progeria treatment breakthrough - ImmInst.org Forums 17 January 2010 4:58 UTC www.imminst.org [Source type: FILTERED WITH BAYES]

.This also shows that it is the buildup of prelamin A in the wrong place, rather than the loss of the normal function of lamin A, that causes the disease.^ In order to block normal cell function and cause Progeria, a molecule called a farnesyl group must be attached to the progerin protein.
  • Progeria.be - Information 17 January 2010 4:58 UTC www.progeria.be [Source type: FILTERED WITH BAYES]

^ Lamin A is normally synthesized as a precursor molecule (prelamin A).
  • Hutchinson-Gilford Progeria Syndrome -- GeneReviews -- NCBI Bookshelf 17 January 2010 4:58 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.
Confocal microscopy photographs of the descending aortas of two 15-month-old progeria mice, one untreated (left picture) and the other treated with the farnsyltransferase inhibitor drug tipifarnib (right picture).
^ Confocal microscopy photographs of the descending aortas of two 15-month-old progeria mice, one untreated (left picture) and the other treated with the farnsyltransferase inhibitor drug tipifarnib (right picture).
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

^ TARA BROWN: Leslie hopes the drug will be tested within months, and every child with Progeria will be offered the chance to take part in the clinical trial.

^ One of the authors, Leslie Gordon, was a physician who didn’t know anything about progeria until her own son, Sam, was diagnosed at 21 months.
  • Progeria | Find Me A Cure 17 January 2010 4:58 UTC findmeacure.com [Source type: FILTERED WITH BAYES]

.The microphotographs show prevention of the vascular smooth muscle cell loss that is otherwise rampant by this age.^ They use high-calorie diets to prevent weight loss and physical therapy but why some people in the forum use calorie restriction to prevent aging ?
  • Progeria treatment breakthrough - ImmInst.org Forums 17 January 2010 4:58 UTC www.imminst.org [Source type: FILTERED WITH BAYES]

Staining was smooth muscle alpha-actin (green), lamins A/C (red) and DAPI (blue). (Original magnification, x 40)
It was hypothesized that part of the reason that treatment with an FFI such as alendronate is inefficient is due to prenylation by geranylgeranyltransferase. Since statins inhibit geranylgeranyltransferase, the combination of an FFI and statins was tried, and markedly improved "the aging-like phenotypes of mice in the metalloproteinase ZMPSTE24, including growth retardation, loss of weight, lipodystrophy, hair loss, and bone defects".[32]

Popular culture

The 1922 short story "The Curious Case of Benjamin Button" by F. Scott Fitzgerald (and later released as a feature film in 2008) may have been inspired by progeria. .The main character, Benjamin Button, is born as a seventy-year-old man and ages backwards.^ Werner syndrome is a form of premature aging (Progeria) which begins during adolescence or early adulthood and results in the appearance of old age by the age of 30 to 40 years.
  • The Progeria Project Foundation - Progeria? - What is Progeria? 17 January 2010 4:58 UTC www.progeriaproject.com [Source type: Academic]

^ Nine year old American boy, Ory Barnett got a heart attact at the age of 5.
  • Progeria.be - Information 17 January 2010 4:58 UTC www.progeria.be [Source type: FILTERED WITH BAYES]

^ According to reports in the medical literature, affected children who are 10 years of age typically have a height approximately that of an average three-year-old child.
  • The Progeria Project Foundation - Progeria? - What is Progeria? 17 January 2010 4:58 UTC www.progeriaproject.com [Source type: Academic]

[33]
.The Hindi film Paa, released in December 2009, has its story line around progeria (starring Amitabh Bachchan playing a thirteen year old boy Auro).^ Amitabh Bachchan is 67 years old.

^ Bachchan was the 'paa' of all makeovers in 2009 .
  • Bachchan was the 'paa' of all makeovers in 2009- Hindustan Times 17 January 2010 4:58 UTC www.hindustantimes.com [Source type: FILTERED WITH BAYES]

^ Amitabh Bachchan’s film ‘Paa’ has brought the problem of Progeria into limelight.
  • Progeria Disease - Mental Retardation - Genetic Mutation - Genetic Disorder » Causes and Symptoms of Progeria 17 January 2010 4:58 UTC www.ayushveda.com [Source type: General]

[34]
.Progeria is also a central theme in the animated film Renaissance in which one of the characters finds the much sought cure.^ Finding the cure for Progeria may also help millions of adults with heart disease and other aging-related conditions.
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC health.kosmix.com [Source type: FILTERED WITH BAYES]
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC www.kosmix.com [Source type: Academic]

[citation needed]
Orlando Gardiner of the science fiction book series Otherland suffers from this disease, and ultimately succumbs to it at the age of 14.
On the episode "Designate This" from the series "Dark Angel", Max's "younger version" has the disease.
.Rabbi Harold Kushner, author of the well known book, "When Bad Things Happen to Good People," had a son who suffered from this disease, and likely his son's tragic illness was the impetus for this book on suffering.^ Rabbi Kushner's book, "When Bad Things Happen to Good People," was inspired by his family's experiences.
  • Dearth of patients makes progeria trial uncertain 17 January 2010 4:58 UTC www.post-gazette.com [Source type: FILTERED WITH BAYES]

^ Gordon and Dr. Berns met with Harold and Suzette Kushner, whose son, Aaron, died of progeria in 1977 at the age of 14.
  • Dearth of patients makes progeria trial uncertain 17 January 2010 4:58 UTC www.post-gazette.com [Source type: FILTERED WITH BAYES]

^ Before Aaron died, Rabbi Kushner recalls, he told his parents he wanted to do three things: visit the National Baseball Hall of Fame and Museum, see an R-rated movie, and meet another child who had progeria.
  • Dearth of patients makes progeria trial uncertain 17 January 2010 4:58 UTC www.post-gazette.com [Source type: FILTERED WITH BAYES]

.The character J.F. Sebastian in the science fiction film Blade Runner states that he suffers from "Methuselah Syndrome," clearly a form of progeria.^ He suffers from a progeria-like syndrome.

^ Werner syndrome is a form of premature aging (Progeria) which begins during adolescence or early adulthood and results in the appearance of old age by the age of 30 to 40 years.
  • The Progeria Project Foundation - Progeria? - What is Progeria? 17 January 2010 4:58 UTC www.progeriaproject.com [Source type: Academic]

^ During his visit to the United States, three other children, also suffering from Progeria, met with him.

[citation needed]
.Progeria was featured in the X-Files episode "Young at Heart," where a scientist studying the disease found a way to reverse the aging process.^ That cellular instability appears to lead to the process of premature aging in Progeria.
  • Progeria.be - Information 17 January 2010 4:58 UTC www.progeria.be [Source type: FILTERED WITH BAYES]

^ Hutchinson-Gilford progeria syndrome (HGPS) is characterized by clinical features that develop in childhood and resemble some features of accelerated aging.
  • Hutchinson-Gilford Progeria Syndrome -- GeneReviews -- NCBI Bookshelf 17 January 2010 4:58 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ As with any person suffering from heart disease, the common events for Progeria children are high blood pressure, strokes, angina (chest pain due to poor blood flow to the heart itself), enlarged heart, and heart failure, all conditions associated with aging.
  • Progeria.be - Information 17 January 2010 4:58 UTC www.progeria.be [Source type: FILTERED WITH BAYES]

.A disease similar to progeria, called Werner syndrome, was featured in the movie Jack (film), starring Robin Williams, in which his character aged 4 times faster than normal.^ Progeria is a very rare genetic disease that causes the body to age at least 8 times faster than normal.

^ Werner syndrome Neurofibromatosis Rare diseases .
  • Progeria - Kosmix : Reference, Videos, Images, News, Shopping and more... 17 January 2010 4:58 UTC www.kosmix.com [Source type: Academic]

^ Since she was born, she's been aging eight times faster than normal.

.Leon Botha, who appeared in a video for the South African conceptual rave-rap group Die Antwoord, has Progeria Syndrome.^ This is a phenomenal success story: Progeria is the premature aging genetic disorder afflicting children, who die of heart disease at an average age of 13 ...

^ Children with progeria, also known as Hutchinson-Gilford progeria syndrome (HGPS), generally appear normal at birth.
  • Progeria 17 January 2010 4:58 UTC www.cnn.com [Source type: General]
  • Progeria 17 January 2010 4:58 UTC edition.cnn.com [Source type: General]
  • OhioHealth - Progeria 17 January 2010 4:58 UTC www.ohiohealth.com [Source type: General]

^ The couple were going to hold their own fund-raiser, in memory of their son, Kristian, who died of a progeria syndrome last year, at age 3.
  • Dearth of patients makes progeria trial uncertain 17 January 2010 4:58 UTC www.post-gazette.com [Source type: FILTERED WITH BAYES]

[35]
The Chuck Palahniuk novel Haunted includes one character, Brandon Whittier, who is dying of progeria at 13.

See also

References

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  31. ^ Loren G. Fong (March 17, 2006). "A Protein Farnesyltransferase Inhibitor Ameliorates Disease in a Mouse Model of Progeria". Science 311 (5767): 1621–3. doi:10.1126/science.1124875. PMID 16484451. http://www.sciencemag.org/cgi/content/abstract/1124875v1. 
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  34. ^ http://www.paathefilm.com/
  35. ^ http://www.youtube.com/watch?v=8NZBxwjcwMc&feature=player_embedded

External links


Citable sentences

Up to date as of December 28, 2010

Here are sentences from other pages on Progeria, which are similar to those in the above article.








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