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货号： bs-0553R 基本售价： 1380.0 元 规格： 100ul
- 规格：100ul
- 价格：1380.00元
- 规格：200ul
- 价格：2200.00元
产品信息
- 产品编号
- bs-0553R
- 英文名称
- Collagen VI alpha 1
- 中文名称
- 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/1380元购买 200ul/2200元购买 大包装/询价
- 说 明 书
- 100ul 200ul
- 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
- WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 106kDa
- 细胞定位
- 细胞浆
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Collagen VI:951-1028/1028
- 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.
- PubMed
- PubMed
- 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.
SWISS:
P12109
Gene ID:
1291
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.
- 产品图片
Sample:
NIH/3T3(Mouse) Cell Lysate at 30 ug
Primary: Anti-Collagen VI alpha 1 (bs-0553R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size:106 kD
Observed band size: 131 kD
Paraformaldehyde-fixed, paraffin embedded (human liver carcinoma); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Collagen VI alpha 1) Polyclonal Antibody, Unconjugated (bs-0553R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Tissue/cell: The skin of the infant; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-Collagen-VI-alpha1 Polyclonal Antibody, Unconjugated(bs-0553R) 1:100, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining