| 產(chǎn)品編號(hào) | bsm-61026R |
| 英文名稱 | Collagen VI Recombinant Rabbit mAb |
| 中文名稱 | 6型膠原重組兔單抗 |
| 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. |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Recombinant |
| 克 隆 號(hào) | 4D10 |
| 交叉反應(yīng) | Human,Mouse,Rat |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 106 kDa |
| 檢測(cè)分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 |
| 性 狀 | Liquid |
| 免 疫 原 | A synthesized peptide derived from human Collagen VI: 15-250 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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. SWISS: P12111 Gene ID: 1293 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 |
| 產(chǎn)品圖片 |
25 ug total protein per lane of various lysates (see on figure) probed with Collagen VI monoclonal antibody, unconjugated (bsm-61026R) at 1:1000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
Paraformaldehyde-fixed, paraffin embedded Human Heart; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Collagen VI Monoclonal Antibody, Unconjugated(bsm-61026R) at 1:300 overnight at 4°C, followed by conjugation to the SP Kit(Rabbit, SP-0023) and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Human Placenta; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Collagen VI Monoclonal Antibody, Unconjugated(bsm-61026R) at 1:300 overnight at 4°C, followed by conjugation to the SP Kit(Rabbit, SP-0023) and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Human Esophagus; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Collagen VI Monoclonal Antibody, Unconjugated(bsm-61026R) at 1:300 overnight at 4°C, followed by conjugation to the SP Kit(Rabbit, SP-0023) and DAB (C-0010) staining.
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