Rabbit Anti-Collagen VI alpha 2/FITC Conjugated antibody

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CO6A2_HUMAN; COL6A2; COL6A2; Collagen alpha 2(VI) chain; Collagen alpha-2(VI) chain; Collagen VI alpha 2 polypeptide; PP3610.

Cat:

SL13963R-FITC

Species Reactivity：

(predicted: Human,Mouse,Dog,Pig,Cow,Horse,)

Immunogen：

KLH conjugated synthetic peptide derived from human Collagen VI alpha 2

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：

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

Host：

Rabbit

Calculated MW：

109kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
Collagen VI acts as a cell-binding protein.

Subcellular Location:
Secreted > extracellular space > extracellular matrix. Membrane. Recruited on membranes by CSPG4.

Post-translational modifications:
Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.

DISEASE:
Defects in COL6A2 are a cause of Bethlem myopathy (BM) [MIM:158810]. 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) [MIM:254090]; 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) [MIM:255600]; 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.

Similarity:
Belongs to the type VI collagen family.
Contains 3 VWFA domains.

Database links:

Entrez Gene: 1292 Human

Entrez Gene: 12834 Mouse

Omim: 12048 Human

SwissProt: P12110 Human

SwissProt: Q02788 Mouse

Unigene: 420269 Human

Unigene: 1949 Mouse

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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