DMPK Mouse anti-Human, Unlabeled, Clone: 17, BD
Mouse Monoclonal Antibody
Manufacturer: BD Biosciences 611569
Myotonic dystrophy (DM), characterized by myotonia and progressive muscle weakness, results from an expanding CTG trinucleotide repeat in the 3′ untranslated region of DMPK, a putative Ser/Thr kinase from the Rho-associated enzyme family. DMPK (myotonic dystrophy protein kinase) is a cytosolic protein that is found in many tissues, especially skeletal and cardiac muscle. Overexpression of DMPK in lens epithelial cells leads to apoptotic-like membrane blebbing and reorganization of the actin cytoskeleton. This effect is similar to that displayed after overexpression of RhoA in epithelial cells. The mutation of DMPK in DM yields decreases DMPK protein levels via increased nuclear retention of DMPK transcripts. Since DMPK knockout mice do not exhibit the extreme phenotype of DM patients, other factors are likely to be involved. MKBP (myotonic dystrophy protein kinase binding protein) is a small heat shock protein that specifically associates with and activates DMPK and protects it from heat-induced inactivation. Thus, DMPK association with MKBP may be important for phospho-regulation of a variety of DMPK substrates, such as cytoskeletal and membrane proteins.
Host Species: Mouse
Species Reactivity: Human
Immunogen: Human DMPK aa. 390-500
Formula Weight [Chemical]: 88kDa
Immunofluorescence, Western Blotting
|Immunofluorescence, Western Blot|
|Human DMPK aa. 390-500|
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