This antibody gave a positive signal in the following tissue lysates: Human Skeletal Muscle; Human Skin; Human Heart as well as the following whole cell lysates: MEF1; NIH3T3; STO; WI38.
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The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 µg/ml. Detects a band of approximately 147 kDa (predicted molecular weight: 108 kDa).
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.
Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
Secreted > extracellular space > extracellular matrix.
Human mRNA for collagen VI alpha 2 C terminal globular domain antibody
Anti-Collagen VI antibody 图像
Western blot - Anti-Collagen VI antibody (ab99249)
All lanes : Anti-Collagen VI antibody (ab99249) at 1 µg/ml
Lane 1 : Human skeletal muscle tissue lysate - total protein (ab29330) Lane 2 : Human thymus tissue lysate - total protein (ab30146) Lane 3 : Human heart tissue lysate - total protein (ab29431) Lane 4 : MEF1 (Mouse embryonic fibroblast cell line) Whole Cell Lysate Lane 5 : NIH 3T3 (Mouse embryonic fibroblast cell line) Whole Cell Lysate Lane 6 : STO (Mouse embryonic fibroblast cell line) Whole Cell Lysate Lane 7 : WI-38 whole cell lysate (ab3960)
Lysates/proteins at 10 µg per lane.
Secondary Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution Developed using the ECL technique