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Matrix Gla protein
Symbol(s): MGP
Genetic data
Locus: Chr. 12 p13.1-12.3
Gene code: MGP
Gene type: protein coding
Protein Structure/Function
Protein length: 84 (Amino Acids)
Other
Covalent modifications: carboxylation
Medical/Biotechnological data
Diseases: Keutel syndrome
Database Links
Entrez: 4256
OMIM: 154870
RefSeq: NM_000900
UniProt: P08493

Matrix gla protein (MGP) is a protein found in numerous body tissues that requires vitamin K for its optimum function. It is present in bone (together with the related vitamin K-dependent protein osteocalcin), as well as in heart, kidney and lung. In bone, its production is increased by vitamin D.

Contents

Genetics

The MGP was linked to the short arm of chromosome 12 in 1990.[1]

Physiology

MGP and osteocalcin are both calcium-binding proteins that may participate in the organisation of bone tissue. Both have glutamate residues that are post-translationally carboxylated by the enzyme gamma-glutamyl carboxylase in a reaction that requires Vitamin K hydroquinone. This process also occurs with a number of proteins involved in coagulation: prothrombin, factor VII, factor IX and factor X, protein C, protein S and protein Z.

Role in disease

Abnormalities in the MGP gene have been linked with Keutel syndrome, a rare condition characterised by abnormal calcium deposition in cartilage, peripheral stenosis of the pulmonary artery, and midfacial hypoplasia.[2]

Mice that lack MGP develop to term but die within two months as a result of arterial calcification which leads to blood-vessel rupture.[3]

References

  1. ^ Cancela L, Hsieh CL, Francke U, Price PA (1990). "Molecular structure, chromosome assignment, and promoter organization of the human matrix Gla protein gene". J. Biol. Chem. 265 (25): 15040–8. PMID 2394711. http://www.jbc.org/cgi/reprint/265/25/15040.  
  2. ^ Munroe PB, Olgunturk RO, Fryns JP, et al. (1999). "Mutations in the gene encoding the human matrix Gla protein cause Keutel syndrome". Nat. Genet. 21 (1): 142–4. doi:10.1038/5102. PMID 9916809.  
  3. ^ Luo G, Ducy P, McKee MD, et al. (March 1997). "Spontaneous calcification of arteries and cartilage in mice lacking matrix GLA protein". Nature 386 (6620): 78–81. doi:10.1038/386078a0. PMID 9052783.  
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