ELISA/assay

Mouse Collagen Type lV ELISA Kit

MBS045436

96 Strip Wells

600 USD

ELISA Kit

Mouse Collagen Type lV ELISA Kit

Collagen Type lV

collagen alpha-1(VI) chain; Collagen alpha-1(VI) chain; collagen alpha-1(VI) chain; collagen alpha-1(VI) chain; alpha 1 (VI) chain (61 AA); collagen VI, alpha-1 polypeptide; collagen, type VI, alpha 1

COL4

COL6A1; COL6A1; OPLL

CO6A1_HUMAN

Mouse

1028

Store all reagents at 2-8 degree C

ELISA Type: Sandwich

87196339

NP_001839.2

NM_001848.2

P12109

108,529 Da

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

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 structure: 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. Post-translational modification: Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains. Involvement in 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. Ref.13 Ref.14 Ref.16 Ref.17Ullrich 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. Ref.15 Ref.16 Ref.18. Sequence similarities: Belongs to the type VI collagen family.Contains 3 VWFA domains.

96 Strip Wells

600 USD