ELK Biotechnology COL6A3 rabbit pAb
COL6A3 단백질을 인식하는 토끼 다클론 항체로, IHC, IF, ELISA에 적합합니다. 인간 및 생쥐 시료에 반응하며, 고순도 IgG 형식으로 제공됩니다. -20°C에서 1년간 안정적으로 보관 가능합니다.
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제품명
COL6A3 rabbit pAb
제품 정보
| 항목 | 내용 |
|---|---|
| Alternative Names | COL6A3; Collagen alpha-3(VI) chain |
| Applications | IHC; IF; ELISA |
| Recommended Dilutions | IHC: 1/100–1/300 IF: 1/200–1/1000 ELISA: 1/40000 Not yet tested in other applications |
| Immunogen | The antiserum was produced against synthesized peptide derived from human Collagen VI alpha3 (AA range: 2261–2310) |
| Host | Rabbit |
| Storage | -20°C, 1 year |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1 mg/ml |
| GeneID (Human) | 1293 |
| Human Swiss-Prot No. | P12111 |
| Cellular Localization | Secreted, extracellular space, extracellular matrix |
| Species Reactivity | Human; Mouse |
Background
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.
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