• Target：
• COL6A2

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

• Gene Name：
• COL6A2

• Protein Name：
• Collagen alpha-2(VI) chain

• Human Gene Id：
• 1292

• Human Swiss Prot No：
• P12110

• Mouse Gene Id：
• 12834

• Mouse Swiss Prot No：
• Q02788

• Immunogen：
• The antiserum was produced against synthesized peptide derived from human Collagen VI alpha2. AA range:691-740

• Specificity：
• COL6A2 Polyclonal Antibody detects endogenous levels of COL6A2 protein.

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

• Source：
• Polyclonal, Rabbit,IgG

• Dilution：
• WB 1:500 - 1:2000. IHC 1:100 - 1:300. IF 1:200 - 1:1000. ELISA: 1:10000. Not yet tested in other applications.

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

• Concentration：
• 1 mg/ml

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

• Other Name：
• COL6A2;Collagen alpha-2(VI) chain

• Observed Band(KD)：
• 109kD

• Background：
• This gene encodes one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective tissues. The product of this gene contains several domains similar to von Willebrand Factor type A domains. 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 this gene are associated with Bethlem myopathy and Ullrich scleroatonic muscular dystrophy. Three transcript variants have been identified for this gene. [provided by RefSeq, Jul 2008],

• Function：
• disease:Defects in COL6A2 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 COL6A2 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.,similarity:Belongs to the type VI collagen family.,similarity:Contains 3

• Subcellular Location：
• Secreted, extracellular space, extracellular matrix . Membrane ; Peripheral membrane protein . Recruited on membranes by CSPG4.

• Expression：
• Fibroblast,Kidney,Liver,Ovary,Placenta,Uterus,

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