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Neurofibrillary tangle: Wikis

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Microscopy image of a neurofibrillary tangle, conformed by hyperphosphorylated tau protein

Neurofibrillary tangles are pathological protein aggregates found within neurons in cases of Alzheimer's disease. They were first described by Alois Alzheimer in one of his patients suffering from the disorder (at the time it was not called Alzheimer's disease). Tangles are formed by hyperphosphorylation of a microtubule-associated protein known as tau, causing it to aggregate in an insoluble form. (These aggregations of hyperphosphorylated tau protein are also referred to as PHF, or "paired helical filaments"). The precise mechanism of tangle formation is not completely understood, and it is still controversial whether tangles are a primary causative factor in the disease or play a more peripheral role.

Other conditions in which neurofibrillary tangles are commonly observed include:

References

  1. ^ Williams DR, Lees AJ. (2009)Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol.8:270-9.
  2. ^ Roberts GW. (1988) Immunocytochemistry of neurofibrillary tangles in dementia pugilistica and Alzheimer's disease. Lancet.2:1456-8.
  3. ^ Selkoe DJ, Podlisny MB (2002) Deciphering the genetic basis of Alzheimer's disease. Annu Rev Genomics Hum Genet 3: 67-99.
  4. ^ Hof PR, et al.(1994) Amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam. Acta Neuropathol. 88:397-404.
  5. ^ Brat DJ, Gearing M, Goldthwaite PT, Wainer BH, Burger PC. (2001) Tau-associated neuropathology in ganglion cell tumours increases with patient age but appears unrelated to ApoE genotype. Neuropathol Appl Neurobiol 27:197-205
  6. ^ Halper J, Scheithauer BW, Okazaki H, Laws ER Jr. (1986) Meningio-angiomatosis. J Neuropathol Exp Neurol. 1986 Jul;45(4):426-46.
  7. ^ Paula-Barbosa MM, Brito R, Silva CA, Faria R, Cruz C.(1979) Neurofibrillary changes in the cerebral cortex of a patient with subacute sclerosing panencephalitis (SSPE). Acta Neuropathol 48:157-60
  8. ^ Wisniewski K, Jervis GA, Moretz RC, Wisniewski HM.(1979) Alzheimer neurofibrillary tangles in diseases other than senile and presenile dementia.Ann Neurol 5:288-94

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