Anti-Collagen VI Rabbit mAb

Product

Anti-Collagen VI Rabbit mAb

Catalog number :AT0711

Collagen VI acts as a cell-binding protein.Defects in COL6A1 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 COL6A1 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.

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

UniProt ID:

P12109

Overview

Reactivity: Human, mouse, rat, bovine, chicken, Drosophilia melanogaster

Tested applications: Western Blotting 1:1000

Immunofluorescence 1:500

Immunohistochemistry (Paraffin) 1:500

Immunoprecipitation(IP) 1;100

Optimal dilutions/concentrations should be determined by the end user.

Specificity: This antibody detects endogenous levels of total Collagen VI protein.

Properties

Immunogen: Full length native protein (purified) corresponding to Human Collagen VI aa 1-1028. Collagen Type VI from human and bovine placenta.

Clonality: Monoclonal, clone number: 6D7

Isotype: Rabbit IgG

Form: Liquid, 100 μl,1mg/ml, PBS (pH 7.2) and 40% Glycerol，0.02% Sodium Azide

Storage instruction: Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C, Avoid freeze / thaw cycle.

Host: Rabbit

Applications

WB Image: Western Blot: Collagen VI Antibody - Analysis of Collagen VI on different lysates using anti-Collagen VI antibody at 1/1,000 dilution.

Lane 1: Mouse heart

Lane 2: Human heart

Lane 3: Human kidney

IHC Image: Immunohistochemistry-Paraffin: Collagen VI Antibody - Analysis of paraffin-embedded mouse colon tissue using anti-Collagen VI antibody. Counter stained with hematoxylin.

ICC/IF Image: Immunocytochemistry/Immunofluorescence: Collagen VI Antibody - Staining Collagen VI in RH-35 cells (green). The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS.

Anti-Collagen VI Rabbit mAb

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