product summary
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company name :
MyBioSource
product type :
antibody
product name :
COL6A2 Polyclonal Antibody
catalog :
MBS128717
quantity :
0.05 mL
price :
285 USD
clonality :
polyclonal
host :
rabbit
conjugate :
nonconjugated
reactivity :
human, mouse, rat
application :
western blot, immunohistochemistry
more info or order :
product information
catalog number :
MBS128717
products type :
Antibody
products full name :
COL6A2 Polyclonal Antibody
products short name :
COL6A2
products name syn :
PP3610
other names :
Collagen alpha-2(VI) chain; Collagen alpha-2(VI) chain; collagen alpha-2(VI) chain; collagen alpha-2(VI) chain; collagen VI, alpha-2 polypeptide; human mRNA for collagen VI alpha-2 C-terminal globular domain; collagen, type VI, alpha 2
products gene name :
COL6A2
other gene names :
COL6A2; COL6A2; PP3610
uniprot entry name :
CO6A2_HUMAN
clonality :
Polyclonal
isotype :
IgG
host :
Rabbit
reactivity :
Human, Mouse, Rat
sequence length :
1019
purity :
Affinity Purification
concentration :
1mg/ml
storage stability :
Store at -20 degree C (regular) or -80 degree C (long term). Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
tested application :
Western Blot (WB), Immunohistochemistry (IHC)
app notes :
WB: 1:200-1:2000. IHC: 1:50-1:200
other info1 :
Species: Human. Immunogen: Recombinant Protein
other info2 :
Immunogen: Recombinant protein of human COL6A2. Calculated Molecular Weight: 109kDa
products categories :
Polyclonal
products description :
This gene encodes one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective tissues. The product of this gene contains several domains similar to von Willebrand Factor type A domains. These domains have been shown to bind extracellular matrix proteins, an interaction that explains the importance of this collagen in organizing matrix components. Mutations in this gene are associated with Bethlem myopathy and Ullrich scleroatonic muscular dystrophy. Three transcript variants have been identified for this gene.
ncbi gi num :
125987812
ncbi acc num :
P12110.4
uniprot acc num :
P12110
ncbi mol weight :
1019
ncbi pathways :
Assembly Of Collagen Fibrils And Other Multimeric Structures Pathway (730306); Axon Guidance Pathway (105688); Collagen Biosynthesis And Modifying Enzymes Pathway (645289); Collagen Formation Pathway (645288); Developmental Biology Pathway (477129); ECM-receptor Interaction Pathway (83068); ECM-receptor Interaction Pathway (479); Extracellular Matrix Organization Pathway (576262); Focal Adhesion Pathway (198795); Focal Adhesion Pathway (83067)
ncbi summary :
This gene encodes one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective tissues. The product of this gene contains several domains similar to von Willebrand Factor type A domains. These domains have been shown to bind extracellular matrix proteins, an interaction that explains the importance of this collagen in organizing matrix components. Mutations in this gene are associated with Bethlem myopathy and Ullrich scleroatonic muscular dystrophy. Three transcript variants have been identified for this gene. [provided by RefSeq, Jul 2008]
uniprot summary :
COL6A2: Collagen VI acts as a cell-binding protein. Defects in COL6A2 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 COL6A2 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. Defects in COL6A2 are the cause of myosclerosis autosomal recessive (MYOSAR); also known as myosclerotic myopathy or congenital myosclerosis of Lowenthal. A condition characterized by chronic inflammation of skeletal muscle with hyperplasia of the interstitial connective tissue. The clinical picture includes slender muscles with firm woody consistency and restriction of movement of many joints because of muscle contractures. Belongs to the type VI collagen family. 3 isoforms of the human protein are produced by alternative splicing. Protein type: Secreted, signal peptide; Secreted; Extracellular matrix. Chromosomal Location of Human Ortholog: 21q22.3. Cellular Component: extracellular matrix; proteinaceous extracellular matrix; extracellular space; collagen; protein complex; endoplasmic reticulum lumen; extracellular region; vesicle; sarcolemma. Molecular Function: protein binding. Biological Process: axon guidance; collagen catabolic process; extracellular matrix disassembly; extracellular matrix organization and biogenesis; response to glucose stimulus; cell adhesion. Disease: Bethlem Myopathy; Myosclerosis, Autosomal Recessive; Ullrich Congenital Muscular Dystrophy
size1 :
0.05 mL
price1 :
285 USD
size2 :
0.2 mL
price2 :
555
more info or order :
company information
MyBioSource
P.O. Box 153308
San Diego, CA 92195-3308
San Diego, CA 92195-3308
sales@mybiosource.com
https://www.mybiosource.com1-888-627-0165
headquarters: USA
MyBioSource, LLC was orginally founded in Vancouver by three enthusiastic scientists who are passionate about providing the world with the best reagents available. Together, they form a company with a big vision known as MyBioSource. MyBioSource is now located in San Diego, California, USA.
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