Proteoglycan, Fetal Cartilage (PRG4) Antibody – Mouse Monoclonal

The protein encoded by this gene is a large proteoglycan specifically synthesized by chondrocytes located at the surface of articular cartilage, and also by some synovial lining cells. This protein contains both chondroitin sulfate and keratan sulfate glycosaminoglycans. It functions as a boundary lubricant at the cartilage surface and contributes to the elastic absorption and energy dissipation of synovial fluid. Mutations in this gene result in camptodactyly-arthropathy-coxa vara-pericarditis syndrome. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

PRG4: Plays a role in boundary lubrication within articulating joints. Prevents protein deposition onto cartilage from synovial fluid by controlling adhesion-dependent synovial growth and inhibiting the adhesion of synovial cells to the cartilage surface. Defects in PRG4 are the cause of camptodactyly- arthropathy-coxa vara-pericarditis syndrome (CACP); also known as Jacobs syndrome. CACP is an autosomal recessive disorder. Individuals with CACP have normal appearing joints at birth but with advancing age develop joint failure associated with noninflammatory synoviocyte hyperplasia and subintimal fibrosis of the synovial capsule. 6 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 1q25-q31

Cellular Component: extracellular space

Molecular Function: scavenger receptor activity; polysaccharide binding

Biological Process: receptor-mediated endocytosis; cell proliferation; negative regulation of interleukin-6 biosynthetic process; immune response; regulation of cell proliferation

Disease: Camptodactyly-arthropathy-coxa Vara-pericarditis Syndrome