Cell Biology Antibodies 12

Anti-COL6A1 Antibody (CAB9236)

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SKU:
CAB9236
Product Type:
Antibody
Applications:
WB
Applications:
IHC
Reactivity:
Human
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology

Description

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Antibody Name:Anti-COL6A1 Antibody
Antibody SKU:CAB9236
Antibody Size:20uL, 50uL, 100uL
Application:WB IHC
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 19-250 of human COL6A1 (NP_001839.2).
Application:WB IHC
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:200
Reactivity:Human, Mouse
Positive Samples:SKOV3, HepG2, HT-1080, Mouse heart, Mouse lung, Mouse pancreas
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 19-250 of human COL6A1 (NP_001839.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:AQDE PETP RAVA FQDC PVDL FFVL DTSE SVAL RLKP YGAL VDKV KSFT KRFI DNLR DRYY RCDR NLVW NAGA LHYS DEVE IIQG LTRM PGGR DALK SSVD AVKY FGKG TYTD CAIK KGLE QLLV GGSH LKEN KYLI VVTD GHPL EGYK EPCG GLED AVNE AKHL GVKV FSVA ITPD HLEP RLSI IATD HTYR RNFT AADW GQSR DAEE AISQ TIDT IVDM IKNN VEQV CCSF
Gene ID:1291
Uniprot:P12109
Cellular Location:Secreted, extracellular matrix, extracellular space
Calculated MW:108kDa
Observed MW:108kDa
Synonyms:COL6A1, BTHLM1, OPLL, UCHMD1
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.
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
Anti-COL6A1 Polyclonal Antibody (CAB9236)Western blot analysis of extracts of various cell lines, using COL6A1 antibody (CAB9236) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 1s.
Anti-COL6A1 Polyclonal Antibody (CAB9236)Immunohistochemistry of paraffin-embedded human lung using COL6A1 antibody (CAB9236) at dilution of 1:100 (40x lens).
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