Recombinant Human Collagen alpha-3 (VI) chain (COL6A3), partial | MyBioSource MBS958856 product information

This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.

product summary

company name :

MyBioSource

product type :

protein

product name :

Recombinant Human Collagen alpha-3 (VI) chain (COL6A3), partial

catalog :

MBS958856

quantity :

0.05 mg (E-Coli)

price :

185 USD

product information

catalog number :

MBS958856

products type :

Recombinant Protein

products full name :

Recombinant Human Collagen alpha-3 (VI) chain (COL6A3), partial

products short name :

Collagen alpha-3 (VI) chain (COL6A3), partial

products name syn :

Collagen alpha-3(VI) chain

other names :

collagen alpha-3(VI) chain isoform 1; Collagen alpha-3(VI) chain; collagen alpha-3(VI) chain; collagen alpha-3(VI) chain; collagen VI, alpha-3 polypeptide; collagen, type VI, alpha 3

products gene name :

COL6A3

other gene names :

COL6A3; COL6A3

uniprot entry name :

CO6A3_HUMAN

host :

E Coli or Yeast or Baculovirus or Mammalian Cell

sequence positions :

2733-2878; Partial.

sequence length :

2878

sequence :

PRDLKIVVLMLTGEVPEQQLEEAQRVILQAKCKGYFFVV
LGIGRKVNIKEVYTFASEPNDVFFKLVDKSTELNEEPLM
RFGRLLPSFVSSENAFYLSPDIRKQCDWFQGDQPTKNLV
KFGHKQVNVPNNVTSSPTSNPVTTTKPVT

purity :

>90%

form :

Liquid containing glycerol; lyophilization may be available upon request.

storage stability :

Store at -20 degrees C. For long-term storage, store at -20 degrees C or -80 degrees C. Store working aliquots at 4 degrees C for up to one week. Repeated freezing and thawing is not recommended.

other info1 :

Species: Homo sapiens (Human)

products description :

Collagen VI acts as a cell-binding protein.

ncbi gi num :

55743098

ncbi acc num :

NP_004360.2

ncbi gb acc num :

NM_004369.3

uniprot acc num :

P12111

ncbi mol weight :

40kD

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 (83067); Focal Adhesion Pathway (478)

ncbi summary :

This gene encodes the alpha-3 chain, one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective tissues. The alpha-3 chain of type VI collagen is much larger than the alpha-1 and -2 chains. This difference in size is largely due to an increase in the number of subdomains, similar to von Willebrand Factor type A domains, that are found in the amino terminal globular domain of all the alpha chains. 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 the type VI collagen genes are associated with Bethlem myopathy, a rare autosomal dominant proximal myopathy with early childhood onset. Mutations in this gene are also a cause of Ullrich congenital muscular dystrophy, also referred to as Ullrich scleroatonic muscular dystrophy, an autosomal recessive congenital myopathy that is more severe than Bethlem myopathy. Multiple transcript variants have been identified, but the full-length nature of only some of these variants has been described. [provided by RefSeq, Jun 2009]

uniprot summary :

COL6A3: Collagen VI acts as a cell-binding protein. Defects in COL6A3 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 COL6A3 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. 3 isoforms of the human protein are produced by alternative splicing. Protein type: Extracellular matrix; Secreted; Secreted, signal peptide. Chromosomal Location of Human Ortholog: 2q37. Cellular Component: extracellular matrix; extracellular space; proteinaceous extracellular matrix; endoplasmic reticulum lumen; extracellular region; collagen type VI; vesicle; sarcolemma. Molecular Function: serine-type endopeptidase inhibitor activity. Biological Process: collagen catabolic process; extracellular matrix disassembly; axon guidance; extracellular matrix organization and biogenesis; muscle development; cell adhesion. Disease: Bethlem Myopathy; Ullrich Congenital Muscular Dystrophy; Dystonia 27

size1 :

0.05 mg (E-Coli)

price1 :

185 USD

size2 :

0.2 mg (E-Coli)

price2 :

420

size3 :

0.5 mg (E-Coli)

price3 :

885

size4 :

1 mg (E-Coli)

price4 :

1280