COL4A6 (G1223) polyclonal antibody

Collagens are fibrous, extracellular matrix proteins with high tensile strength and are the major components of connective tissue, such as tendons and cartilage. Collagens contain triple helix domains and frequently show lateral selfassociation in order to form complex connective tissues. Several collagens play a role in cell adhesion, which is important for maintaining normal tissue architecture and function. COL4A6 (collagen, type IV, α 6) is a 1,691 amino acid secreted protein belonging to the type IV collagen family. Type IV collagen, a major component of the basement membrane (BM), is composed of six genetically distinct α(IV) chains, α1(IV) to α6(IV). Defects in the gene encoding COL4A6 is believed is the cause of X-linked Alport syndrome (AS), which is characterized by macroscopic hematuria, cataracts and leiomyomatosis.