Type IV collagen, the major structural component of basement membranes, is a multimeric protein composed of 3 alpha subunits. These subunits are encoded by 6 different genes, alpha 1 through alpha 6, each of which can form a triple helix structure with 2 other subunits to form type IV collagen. COL4A3 encodes alpha 3. In the Goodpasture syndrome, autoantibodies bind to the collagen molecules in the basement membranes of alveoli and glomeruli. The epitopes that elicit these autoantibodies are localized largely to the non-collagenous C-terminal domain of the protein. A specific kinase phosphorylates amino acids in this same C-terminal region and the expression of this kinase is upregulated during pathogenesis. COL4A3 is also linked to an autosomal recessive form of Alport syndrome. The mutations contributing to this syndrome are also located within the exons that encode this C-terminal region. Like the other members of the type IV collagen gene family, COL4A3 is organized in a head-to-head conformation with another type IV collagen gene so that each gene pair shares a common promoter. 蛋白別名為：COL4A3; Collagen alpha-3(IV) chain; Goodpasture antigen;基因ID為：1285；蛋白質ID:Q01955
應用類型
WB，ELISA補充：最優的抗體稀釋比例需要基于客戶實驗進行優化.建議的起始稀釋比例如下: WB: 1:500-1:2000, ELISA: 1:40000. Not yet tested in other applications.
免疫原
合成多肽：the C-terminal region of human Tumstatin. at AA rangle: 1380-1460
