• 靶点：
• COL6A3

• 简介：
• >>PI3K-Akt signaling pathway;>>Focal adhesion;>>ECM-receptor interaction;>>Protein digestion and absorption;>>Human papillomavirus infection

• 基因名称：
• COL6A3

• 蛋白名称：
• Collagen alpha-3(VI) chain

• Human Gene Id：
• 1293

• Human Swiss Prot No：
• P12111

• 免疫原：
• The antiserum was produced against synthesized peptide derived from human Collagen VI alpha3. AA range:2261-2310

• 特异性：
• COL6A3 Polyclonal Antibody detects endogenous levels of COL6A3 protein.

• 组成：
• Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

• 来源：
• Polyclonal, Rabbit,IgG

• 稀释：
• IHC 1:100 - 1:300. IF 1:200 - 1:1000. ELISA: 1:40000. Not yet tested in other applications.

• 纯化工艺：
• The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

• 浓度：
• 1 mg/ml

• 储存：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• 其他名称：
• COL6A3;Collagen alpha-3(VI) chain

• 分子量：
• 344kD

• 背景：
• This gene encodes the alpha-3 chain, one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective tissues. The alpha-3 chain of type VI collagen is much larger than the alpha-1 and -2 chains. This difference in size is largely due to an increase in the number of subdomains, similar to von Willebrand Factor type A domains, that are found in the amino terminal globular domain of all the alpha chains. These domains have been shown to bind extracellular matrix proteins, an interaction that explains the importance of this collagen in organizing matrix components. Mutations in the type VI collagen genes are associated with Bethlem myopathy, a rare autosomal dominant proximal myopathy with early childhood onset. Mutations in this gene are also a cause of Ullrich congenital muscular dystrophy, also referred to as Ullrich scleroatonic muscular dystrophy, an a

• 功能：
• disease:Defects in COL6A3 are a cause of Bethlem myopathy (BM) [MIM:158810]. BM is a rare autosomal dominant proximal myopathy characterized by early childhood onset (complete penetrance by the age of 5) and joint contractures most frequently affecting the elbows and ankles.,disease:Defects in COL6A3 are a cause of Ullrich congenital muscular dystrophy (UCMD) [MIM:254090]; also known as Ullrich scleroatonic muscular dystrophy. UCMD is an autosomal recessive congenital myopathy characterized by muscle weakness and multiple joint contractures, generally noted at birth or early infancy. The clinical course is more severe than in Bethlem myopathy.,function:Collagen VI acts as a cell-binding protein.,PTM:Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.,PTM:The N-terminus is blocked.,similarity:Belongs to the type VI collage

• 细胞定位：
• Secreted, extracellular space, extracellular matrix .

• 组织表达：
• Colon endothel,Fibroblast,Human uterus,Kidney,Liver,Placenta,Plasma,Pooled,

Key genes associated with prognosis and metastasis of clear cell renal cell carcinoma Peerj. 2022 Jan;10:e12493. IHC Human 1:100 Clear cell renal cell carcinoma (ccRCC) tissue
货号：YT1036

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