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Cleaved-COL4A3 (L1425) rabbit pAb
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ES1036
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50?L ￥1280.00
100?L ￥1980.00

Overview

产品名称: Cleaved-COL4A3 (L1425) rabbit pAb
反应性: Human;Rat;Mouse;
又名: COL4A3; Collagen alpha-3(IV) chain; Goodpasture antigen
宿主: Rabbit
稀释比: Western Blot: 1/500 - 1/2000. ELISA: 1/5000. Not yet tested in other applications.
免疫原: The antiserum was produced against synthesized peptide derived from human Collagen IV alpha3. AA range:1376-1425
贮存条件: -20°C/1 year
克隆性: Polyclonal
抗体亚型: IgG
浓度: 1 mg/ml
实测条带: 140kD
基因ID: 1285
卵白序列: Q01955
细胞定位: Secreted, extracellular space, extracellular matrix, basement membrane. Colocalizes with COL4A4 and COL4A5 in GBM, tubular basement membrane (TBM) and synaptic basal lamina (BL). .
研究配景: 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. This gene 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. This gene 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 r
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