Anti-Collagen VI/COL6A1 Antibody (CAB9738)

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SKU:
CAB9738
Product type:
Antibody
Antibody Type:
Monoclonal Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Synonyms:
BTHLM1
Synonyms:
OPLL
Synonyms:
UCHMD1
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Description

抗体名:Collagen VI/COL6A1 Rabbit mAb
抗体コード:CAB9738
サイズ:20uL, 50uL, 100uL
同義語:BTHLM1, OPLL, UCHMD1
申し込み:WB, IHC
反応性:Human, Mouse, Rat
宿主種:Rabbit
免疫原:A synthesized peptide derived from human Collagen VI/COL6A1
申し込み:WB, IHC
推奨される希釈:WB 1:500 - 1:2000 IHC 1:50 - 1:200
反応性:Human, Mouse, Rat
ポジティブサンプル:Mouse lung, Rat heart
免疫原:A synthesized peptide derived from human Collagen VI/COL6A1
精製方法:Affinity purification
保管所:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.
アイソタイプ:IgG
順序:Email for sequence
遺伝子ID:1291
Uniprot:P12109
セルラーロケーション:Secreted, extracellular matrix, extracellular space
計算された分子量:147kDa
観察された分子量:140kDa
UniProt Protein Function:COL6A1: Collagen VI acts as a cell-binding protein. Defects in COL6A1 are a cause of Bethlem myopathy (BM). BM is a rare autosomal dominant proximal myopathy characterized by early childhood onset (complete penetrance by the age of 5) and joint contractures most frequently affecting the elbows and ankles. Defects in COL6A1 are a cause of Ullrich congenital muscular dystrophy (UCMD); also known as Ullrich scleroatonic muscular dystrophy. UCMD is an autosomal recessive 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. Belongs to the type VI collagen family.
UniProt Protein Details:

Protein type:Secreted; Secreted, signal peptide; Extracellular matrix

Chromosomal Location of Human Ortholog: 21q22.3

Cellular Component: endoplasmic reticulum lumen; extracellular matrix; extracellular region; lysosomal membrane; membrane; protein complex

Molecular Function:platelet-derived growth factor binding

Biological Process: collagen catabolic process; extracellular matrix organization and biogenesis; osteoblast differentiation

Disease: Bethlem Myopathy; Ullrich Congenital Muscular Dystrophy

NCBI Summary: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]
UniProt Code:P12109
NCBI GenInfo Identifier:125987811
NCBI Gene ID:1291
NCBI Accession:P12109.3
UniProt Secondary Accession:P12109,O00117, O00118, Q14040, Q14041, Q16258, Q7Z645 Q9BSA8,
UniProt Related Accession:P12109
Molecular Weight:108,529 Da
NCBI Full Name:Collagen alpha-1(VI) chain
NCBI Synonym Full Names:collagen type VI alpha 1 chain
NCBI Official Symbol:COL6A1  
NCBI Official Synonym Symbols:OPLL; BTHLM1; UCHMD1  
NCBI Protein Information:collagen alpha-1(VI) chain
UniProt Protein Name:Collagen alpha-1(VI) chain
Protein Family:Collagen
UniProt Gene Name:COL6A1  
UniProt Entry Name:CO6A1_HUMAN
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