MGP

This gene encodes a member of the osteocalcin/matrix Gla family of proteins. The encoded vitamin K-dependent protein is secreted by chondrocytes and vascular smooth muscle cells, and functions as a physiological inhibitor of ectopic tissue calcification. Carboxylation status of the encoded protein is associated with calcification of the vasculature in human patients with cardiovascular disease and calcification of the synovial membranes in osteoarthritis patients. Mutations in this gene cause Keutel syndrome in human patients, which is characterized by abnormal cartilage calcification, peripheral pulmonary stenosis and facial hypoplasia. [provided by RefSeq, Sep 2016]

Matrix Gla Protein

Associates with the organic matrix of bone and cartilage. Thought to act as an inhibitor of bone formation.

Cartilage condensation Source: ProtInc
Cell differentiation Source: UniProtKB-KW
Ossification Source: ProtInc
Regulation of bone mineralization Source: InterPro

Secreted

Keutel syndrome (KTLS):
An autosomal recessive disorder characterized by abnormal cartilage calcification, peripheral pulmonary stenosis neural hearing loss and midfacial hypoplasia.

Requires vitamin K-dependent gamma-carboxylation for its function.