Anti-Human/Mouse COL4A3 Polyclonal Antibody

COL4A3多克隆抗体

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 region. Like the other members of the type IV collagen gene family, this gene is organized in a head-to-head conformation with another type IV collagen gene so that each gene pair shares a common promoter.
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基本信息

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货号 PA10694
规格 100μl
宿主 Rabbit
免疫原 Synthetic peptide of human COL4A3
特异性 Alpha 3 and alpha 4 type IV collagens are colocalized and present in kidney, eye, basement membranes of lens capsule, cochlea, lung, skeletal muscle, aorta, synaptic fibers, fetal kidney and fetal lung. PubMed:8083201 reports similar levels of expression of alpha 3 and alpha 4 type IV collagens in kidney, but PubMed:7523402 reports that in kidney levels of alpha 3 type IV collagen are significantly lower than those of alpha 4 type IV collagen. According to PubMed:8083201, alpha 3 type IV collagen is not detected in heart, brain, placenta, liver, pancreas, extrasynaptic muscle fibers, endoneurial and perineurial nerves, fetal brain, fetal heart and fetal liver. According to PubMed:7523402, alpha 3 type IV collagen is strongly expressed in pancreas, neuroretina and calvaria and not expressed in adrenal, ileum and skin. Isoform 1 and isoform 3 are strongly expressed in kidney, lung, suprarenal capsule, muscle and spleen, in each of these tissues isoform 1 is more abundant than isoform 3. Isoform 1 and isoform 3 are expressed at low levels in artery, fat, pericardium and peripherical nerve, but not in placenta, mesangium, skin, pleura and cultured umbilical endothelial cells.
亚型 IgG
反应性 Human,Mouse
克隆
Uniprot Q01955
浓度 0.2 mg/mL
推荐稀释度 IHC 1:25-1:100
配方
应用范围 IHC,ELISA
别名 Alpha 3 type IV collagen,Alpha3 type IV collagen,CO4A3,COL4A 3,Col4a3,Collagen alpha 3(IV) chain,Collagen IV alpha 3 polypeptide,Collagen type IV alpha 3 (Goodpasture antigen),Collagen type IV alpha 3,Collagen type IV alpha 3 chain,Goodpasture antigen,OTTHUMP00000195044,Tumstatin
保存条件 冻干产品: 2 - 8°C保存5年; 液体: -20°C保存2年.
注意 仅用于科学研究, 不能用于疾病诊断.

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