| Product Name | Human Collagen alpha-1 (VI) chain (COL6A1) ELISA Kit |
|---|---|
| Description | The collagens are a superfamily of proteins that play a role in maintaining the integrity of various tissues. Collagens are extracellular matrix proteins and have a triple-helical domain as their common structural element. Collagen VI is a major structural component of microfibrils. The basic structural unit of collagen VI is a heterotrimer of the alpha1(VI), alpha2(VI), and alpha3(VI) chains. The alpha2(VI) and alpha3(VI) chains are encoded by the COL6A2 and COL6A3 genes, respectively. The protein encoded by this gene is the alpha 1 subunit of type VI collagen (alpha1(VI) chain). Mutations in the genes that code for the collagen VI subunits result in the autosomal dominant disorder, Bethlem myopathy.Defects in COL6A1 are a cause of Bethlem myopathy (BM). This assay has high sensitivity and excellent specificity for detection of Human COL6A1. No significant cross-reactivity or interference between Human COL6A1 and analogues was observed. |
| Synonyms | OPLL, alpha 1 (VI) chain (61 AA)|collagen VI, alpha-1 polypeptide |
| Method | Sandwich ELISA |
| Detection Range | 62.5-4000 pg/mL |
| Sensitivity | 23.5 pg/mL |
| Reactivity | Human |
| Sample Types | Serum, Plasma, Other biological fluids. |
| Background |
The collagens are a superfamily of proteins that play a role in maintaining the integrity of various tissues. Collagens are extracellular matrix proteins and have a triple-helical domain as their common structural element. Collagen VI is a major structural component of microfibrils. The basic structural unit of collagen VI is a heterotrimer of the alpha1(VI), alpha2(VI), and alpha3(VI) chains. The alpha2(VI) and alpha3(VI) chains are encoded by the COL6A2 and COL6A3 genes, respectively. The protein encoded by this gene is the alpha 1 subunit of type VI collagen (alpha1(VI) chain). Mutations in the genes that code for the collagen VI subunits result in the autosomal dominant disorder, Bethlem myopathy.Defects in COL6A1 are a cause of Bethlem myopathy (BM). |
| Supplier | Abebio |
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