ABclonal Technology COL4A3 Rabbit pAb
Type IV collagen, a major component of basement membranes, is a multimeric protein composed of 3 alpha subunits, encoded by six genes (alpha 1-6). This gene encodes the alpha 3 subunit. In Goodpasture syndrome, autoantibodies target the collagen in basement membranes of alveoli and glomeruli, binding to epitopes in the non-collagenous C-terminal domain. A specific kinase phosphorylates amino acids in this region, and its expression increases during pathogenesis. This gene is also linked to an autosomal recessive form of Alport syndrome, with mutations in the C-terminal region contributing to the disease. Like other type IV collagen genes, this gene is organized in a head-to-head conformation with another type IV collagen gene, sharing a common promoter.