| 產(chǎn)品編號 | bs-0553R-BF350 |
| 英文名稱1 | Rabbit Anti-Collagen VI alpha 1/BF350 Conjugated antibody |
| 中文名稱 | BF350標(biāo)記的抗Ⅵ型膠原抗體 |
| 別 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. |
| 規(guī)格價格 | 100ul/2980元 購買 大包裝/詢價 |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 腫瘤 細(xì)胞生物 信號轉(zhuǎn)導(dǎo) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Rabbit, ) |
| 產(chǎn)品應(yīng)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 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Collagen VI C-terminus |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol |
| 保存條件 | 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. |
| 產(chǎn)品介紹 |
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 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. |
