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抗Ⅵ型胶原抗体
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相关标签：抗Ⅵ型胶原抗体
详细内容
产品编号Rs-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.
说 明 书0.1ml 0.2ml
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=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 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/1ml
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.
PubMedPubMed
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.
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.
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.
说 明 书0.1ml 0.2ml
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=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 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/1ml
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.
PubMedPubMed
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.
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.
抗Ⅵ型胶原抗体
