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Anti-Collagen VI α2 Antibody

品牌：Antibodies

貨號：

規(guī)格：50μl

貨期：

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Anti-Collagen VI α2 Antibody

商品詳情參考文獻相關(guān)資料

Name:	Anti-Collagen VI α2 Antibody See all Collagen VI α2 primary antibodies
Description:	Rabbit polyclonal antibody to Collagen VI α2
Specificity:	The antibody detects endogenous levels of total Collagen VI α2 protein.
Applications:	WB, IHC
Reactivity:	Human, Mouse
Immunogen:	Synthesized peptide derived from internal of human Collagen VI α2.
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. Myosclerosis autosomal recessive: A condition characterized by chronic inflammation of skeletal muscle with hyperplasia of the interstitial connective tissue. The clinical picture includes slender muscles with firm 'woody' consistency and restriction of movement of many joints because of muscle contractures.
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. Membrane. Recruited on membranes by CSPG4.