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Anti-Collagen XII α1 Antibody

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产品名称: Anti-Collagen XII α1 Antibody
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Anti-Collagen XII α1 Antibody


Anti-Collagen XII α1 Antibody  的详细介绍
Name: Anti-Collagen XII α1 Antibody
See all Collagen XII α1 primary antibodies
Description: Rabbit polyclonal antibody to Collagen XII α1
Specificity: The antibody detects endogenous levels of total Collagen XII α1 protein.
Applications: IHC, IF
Reactivity: Human
Immunogen: Synthesized peptide derived from internal of human Collagen XII α1.
Host: Rabbit
Clonality: Polyclonal
Conjugate: Unconjugated
Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration: 1.0mg / ml
Formulation: Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Storage: Store at -20?C
Function: Type XII collagen interacts with type I collagen-containing fibrils, the COL1 domain could be associated with the surface of the fibrils, and the COL2 and NC3 domains may be localized in the perifibrillar matrix.
Tissue Specificity: Found in collagen I-containing tissues: both isoform 1 and isoform 2 appear in amnion, chorion, skeletal muscle, small intestine, and in cell culture of dermal fibroblasts, keratinocytes and endothelial cells. Only isoform 2 is found in lung, placenta, kidney and a squamous cell carcinoma cell line. Isoform 1 is also present in the corneal epithelial Bowman's membrane (BM) and the interfibrillar matrix of the corneal stroma, but it is not detected in the limbal BM.
Involvement in Disease: Ullrich congenital muscular dystrophy 2: A form of Ullrich muscular dystrophy, 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.

Bethlem myopathy 2: A form of Bethlem myopathy, a benign proximal myopathy characterized by early childhood onset and joint contractures most frequently affecting the elbows and ankles.
Sequence Similarities: Belongs to the fibril-associated collagens with interrupted helices (FACIT) family.
Post-Translational Modification: The triple-helical tail is stabilized by disulfide bonds at each end.
Cellular Location: Secreted > Extracellular space > Extracellular matrix.
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