Anti-Collagen XII α1 Antibody |产品详情|进口试剂采购网

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Anti-Collagen XII α1 Antibody

品牌：Antibodies

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规格：50µl

货期：

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Anti-Collagen XII α1 Antibody

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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.