BGN

The protein encoded by this gene is a small cellular or pericellular matrix proteoglycan that is closely related in structure to two other small proteoglycans, decorin and fibromodulin. The encoded protein and decorin are thought to be the result of a gene duplication. Decorin contains one attached glycosaminoglycan chain, while this protein probably contains two chains. For this reason, this protein is called biglycan. This protein is thought to function in connective tissue metabolism by binding to collagen fibrils and transfering growth factor-beta. It may promote neuronal survival. This gene is a candidate gene for the Happle syndrome. [provided by RefSeq]

Full Name

biglycan

Function

May be involved in collagen fiber assembly.

Biological Process

Articular cartilage development Source: Ensembl
Blood vessel remodeling Source: Ensembl
Bone development Source: Ensembl
Chondroitin sulfate biosynthetic process Source: Reactome
Chondroitin sulfate catabolic process Source: Reactome
Dermatan sulfate biosynthetic process Source: Reactome
Extracellular matrix organization Source: Reactome
Peptide cross-linking via chondroitin 4-sulfate glycosaminoglycan Source: Ensembl

Cellular Location

Extracellular matrix

Involvement in disease

Meester-Loeys syndrome (MRLS): An X-linked, thoracic aortic aneurysm syndrome characterized by early-onset, severe aortic aneurysm and dissection. Other recurrent findings include hypertelorism, pectus deformity, joint hypermobility, contractures, and mild skeletal dysplasia.
Spondyloepimetaphyseal dysplasia, X-linked (SEMDX): An X-linked recessive bone disease characterized by severe short-trunk dwarfism, brachydactyly, metaphyseal flaring of lower extremities, short and broad long bone diaphyses, moderate platyspondyly, normal facies, and normal intelligence.

PTM

The two attached glycosaminoglycan chains can be either chondroitin sulfate or dermatan sulfate.