| 词条 | Matrix gla protein |
| 释义 |
MGP is found in number body tissues in mammals, birds, and fish. Its mRNA is present in bone, cartilage, heart, and kidney.[3] It is present in bone together with the related vitamin K2-dependent protein osteocalcin. In bone, its production is increased by vitamin D. GeneticsThe MGP was linked to the short arm of chromosome 12 in 1990.[4] Its mRNA sequence length is 585 bases long in humans.[5] PhysiologyMGP 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 diseaseAbnormalities 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.[6] Mice that lack MGP develop to term but die within two months as a result of arterial calcification which leads to blood-vessel rupture.[2] References1. ^{{cite journal |vauthors=Yao Y, Jumabay M, Ly A, Radparvar M, Cubberly MR, Boström KI | title = A role for the endothelium in vascular calcification | journal = Circ. Res. | volume = 113 | issue = 5 | pages = 495–504 | year = 2013 | pmid = 23852538 | pmc = 3851028 | doi = 10.1161/CIRCRESAHA.113.301792 }} 2. ^1 {{cite journal |vauthors=Luo G, Ducy P, McKee MD, Pinero GJ, Loyer E, Behringer RR, Karsenty G | title = Spontaneous calcification of arteries and cartilage in mice lacking matrix GLA protein | journal = Nature | volume = 386 | issue = 6620 | pages = 78–81 | date = March 1997 | pmid = 9052783 | doi = 10.1038/386078a0 | url = }} 3. ^{{cite journal |vauthors=Pinto JP, Conceição N, Gavaia PJ, Cancela ML | title = Matrix Gla protein gene expression and protein accumulation colocalize with cartilage distribution during development of the teleost fish Sparus aurata | journal = Bone | volume = 32 | issue = 3 | pages = 201–10 | year = 2003 | pmid = 12667547 | doi = 10.1016/S8756-3282(02)00981-X }} 4. ^{{cite journal |vauthors=Cancela L, Hsieh CL, Francke U, Price PA | title = Molecular structure, chromosome assignment, and promoter organization of the human matrix Gla protein gene | journal = J. Biol. Chem. | volume = 265 | issue = 25 | pages = 15040–8 | year = 1990 | pmid = 2394711 | url = http://www.jbc.org/cgi/reprint/265/25/15040 }} 5. ^{{cite web | url = http://www.ebi.ac.uk/ena/data/view/M58549 | title = Sequence: M58549.1 : Human matrix Gla protein (MGP) mRNA, complete cds. | format = | work = European Nucleotide Archive | publisher = European Bioinformatics Institute }} 6. ^{{cite journal |vauthors=Munroe PB, Olgunturk RO, Fryns JP, Van Maldergem L, Ziereisen F, Yuksel B, Gardiner RM, Chung E | title = Mutations in the gene encoding the human matrix Gla protein cause Keutel syndrome | journal = Nat. Genet. | volume = 21 | issue = 1 | pages = 142–4 | year = 1999 | pmid = 9916809 | doi = 10.1038/5102 }} External links
3 : Extracellular matrix proteins|Genes on human chromosome 12|Glycoproteins |
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