Anti-Collagen XVII antibody

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

Collagen XVII also known as collagen 17 or 17a1 collagen is a transmembrane protein that primarily functions as a structural component in epithelial cells. It weighs approximately 180 kDa and is mainly expressed in the basement membrane of stratified epithelia. Collagen XVII forms hemidesmosomes which are essential for the attachment of epithelial cells to the underlying basement membrane. The protein ensures mechanical stability and integrity of tissues where it is found.

Biological function summary

Collagen XVII contributes to the maintenance of cell adhesion and tissue architecture. It acts as a component of the hemidesmosome complex connecting the extracellular matrix to the cytoskeleton through integrins. This interaction promotes structural stability and supports tissue repair processes. The protein's expression reaches high levels in skin mucous membranes and the cornea playing a significant role in maintaining the barrier function of these tissues.

Pathways

Collagen XVII interacts with cellular signaling pathways related to cell adhesion and migration. The protein is involved in the integrin-mediated signaling pathway which regulates cell survival and differentiation. Additionally it participates in the basement membrane pathway collaborating with proteins such as laminin and bullous pemphigoid antigens to maintain tissue cohesion and repair.

Associated diseases and disorders

Collagen XVII is closely associated with bullous pemphigoid and junctional epidermolysis bullosa. In bullous pemphigoid antibodies target collagen XVII leading to skin blistering due to weakened cell adhesion. In junctional epidermolysis bullosa mutations in the gene encoding collagen XVII disrupt its function resulting in fragile skin and mucosa. These disorders highlight the important role this protein plays in maintaining epithelial tissue integrity and function.