FITC标记的抗Ⅵ型胶原抗体
产品名称： FITC标记的抗Ⅵ型胶原抗体
英文名称： Anti-Collagen VI alpha 1/FITC
产品编号： HZ-0553R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： IF=1:50-200
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Rabbit Anti-Collagen VI alpha 1/FITC Conjugated antibody
FITC标记的抗Ⅵ型胶原抗体
| 英文名称 | Anti-Collagen VI alpha 1/FITC |
| 中文名称 | FITC标记的抗Ⅵ型胶原抗体 |
| 别 名 | CO6A1_HUMAN; COL6A1; COL6A2; COL6A3; Collagen alpha 1(VI) chain; Collagen alpha 1(VI) chain precursor; Collagen alpha-1(VI) chain; Collagen type VI alpha 1; Collagen type VI alpha 2; Collagen type VI alpha 3; Collagen VI alpha 1 polypeptide; Collagen VI alpha 2 polypeptide; Collagen VI alpha 3 polypeptide; CollagenVI; Human mRNA for collagen VI alpha 1 C terminal globular domain; OPLL; PP3610. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 细胞生物 信号转导 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Rabbit, |
| 产品应用 | IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 106kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Collagen VI C-terminus |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | 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. [provided by RefSeq, Jul 2008] Function: Collagen VI acts as a cell-binding protein. Subunit: Trimers composed of three different chains: alpha-1(VI), alpha-2(VI), and alpha-3(VI) or alpha-5(VI) or alpha-6(VI). Subcellular Location: Secreted, extracellular space, extracellular matrix (By similarity). Tissue Specificity: Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains. DISEASE: Bethlem myopathy (BM) [MIM:158810]: A benign autosomal dominant proximal myopathy characterized by early childhood onset and joint contractures most frequently affecting the elbows and ankles. Note=The disease is caused by mutations affecting the gene represented in this entry. Ullrich congenital muscular dystrophy (UCMD) [MIM:254090]: UCMD is a 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. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the type VI collagen family. Contains 3 VWFA domains. Database links: Entrez Gene: 1291 Human Entrez Gene: 1292 Human Entrez Gene: 1293 Human Entrez Gene: 12833 Mouse Entrez Gene: 12834 Mouse Entrez Gene: 12835 Mouse Entrez Gene: 294337 Rat Entrez Gene: 361821 Rat Omim: 120220 Human Omim: 120240 Human Omim: 120250 Human SwissProt: P12109 Human SwissProt: P12110 Human SwissProt: P12111 Human SwissProt: Q02788 Mouse SwissProt: Q04857 Mouse Unigene: 474053 Human Unigene: 2509 Mouse Unigene: 232118 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
胶原蛋白是蛋白质的一个超家族，在维持各种组织的完整性中起作用。胶原蛋白是细胞外基质蛋白，具有三螺旋结构域作为其共同的结构元件。胶原蛋白VI是微纤维的主要结构组分。胶原VI的基本结构单元是α1（Ⅵ）、α2（Ⅵ）和α3（Ⅵ）链的异三聚体。AlpHA2（VI）和AlpHA3（Ⅵ）链分别由COL6A2和COL6A3基因编码。由该基因编码的蛋白质是VI型胶原（α1（VI）链）的α1亚基。基因编码的胶原蛋白VI亚基的突变导致常染色体显性遗传疾病，Bethlem myopathy。[ RefSeq，JUL 2008 ]