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Anti-Collagen VI α3 Antibody

品牌：Antibodies

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規(guī)格：50μl

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Anti-Collagen VI α3 Antibody

商品詳情參考文獻(xiàn) 相關(guān)資料

Name:	Anti-Collagen VI α3 Antibody See all Collagen VI α3 primary antibodies
Description:	Rabbit polyclonal antibody to Collagen VI α3
Specificity:	The antibody detects endogenous levels of total Collagen VI α3 protein.
Applications:	IHC, IF
Reactivity:	Human, Mouse
Immunogen:	Synthesized peptide derived from internal of human Collagen VI α3.
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:	Collagen VI acts as a cell-binding protein.
Involvement in Disease:	Bethlem myopathy 1: A benign proximal myopathy characterized by early childhood onset and joint contractures most frequently affecting the elbows and ankles. Ullrich congenital muscular dystrophy 1: 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. Dystonia 27: A form of dystonia, a disorder defined by the presence of sustained involuntary muscle contraction, often leading to abnormal postures. DYT27 is an autosomal recessive form characterized by segmental isolated dystonia involving the face, neck, bulbar muscles, and upper limbs.
Sequence Similarities:	Belongs to the type VI collagen family.
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.
Cellular Location:	Secreted > Extracellular space > Extracellular matrix.