Key features and details
- Expression system: Native
- Suitable for: WB, IP, ELISA
参阅全部 Collagen VI 蛋白酶
蛋白长度Full length protein
Our Abpromise guarantee covers the use of ab7531 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
This product reacts with anti-Collagen Type VI. Reaction with anti-Collagen I, II, III, IV or V is negligible (typically less than 1% cross reactivity was detected by ELISA).
This product is not recommended for use under denaturing conditions in WB, IP, and ELISA. We would suggest testing it under native conditions.
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Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Constituent: 0.82% Sodium acetate
- Alpha 1 (VI) chain (61 AA)
功能Collagen VI acts as a cell-binding protein.
疾病相关Defects in COL6A1 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.
Defects in COL6A1 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.
序列相似性Belongs to the type VI collagen family.
Contains 3 VWFA domains.
翻译后修饰Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
细胞定位Secreted > extracellular space > extracellular matrix.
- Information by UniProt
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.