Rabbit Anti-Collagen VI alpha 1/FITC Conjugated antibody

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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 a

Cat:

SL0553R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Rabbit,)

Immunogen：

KLH conjugated synthetic peptide derived from human Collagen VI C-terminus

Format：

Lyophilized or Liquid

Storage instructions：

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 ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

106kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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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.

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: 12048 Human

Omim: 120250 Human

SwissProt: P12109 Human