• Desmin (ABT479) Mouse mAb
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Upingbio

YP-Ab-15165-53UL

Desmin (ABT479) Mouse mAb

Desmin (ABT479) Mouse mAb

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  • Reaction species: Human;Mouse;Rat
  • Gene Name: DES
  • Protein name: CMD1I;CSM1;CSM2;DES;DESM_HUMAN;Desmin;FLJ12025;FLJ39719;FLJ41013;FLJ41793;Intermediate filament protein;OTTHUMP00000064865
  • Immunogen: Synthesized peptide derived from human Desmin
  • Specificity: The antibody can specifically recognize human Desmin protein.
  • Composition: PBS, pH7.2, 0.03% Porcolin 300, containing stabilizing protein
  • Source: Monoclonal Mouse IgG2b, kappa
  • Dilution ratio: IHC-p 1:200-400, WB 1:200-1000,
  • Purification process: The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen.
  • Storage: -20°C
  • Other Names: CMD1I;CSM1;CSM2;DES;DESM_HUMAN;Desmin;FLJ12025;FLJ39719;FLJ41013;FLJ41793;Intermediate filament protein;OTTHUMP00000064865
  • Background: This gene encodes a muscle-specific class III intermediate filament. Homopolymers of this protein form a stable intracytoplasmic filamentous network connecting myofibrils to each other and to the plasma membrane. Mutations in this gene are associated with desmin-related myopathy, a familial cardiac and skeletal myopathy (CSM), and with distal myopathies. [provided by RefSeq, Jul 2008],
  • Function: disease:Defects in DES are the cause of cardiomyopathy dilated type 1I (CMD1I) [MIM:604765]. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.,disease:Defects in DES are the cause of desmin-related cardio-skeletal myopathy (CSM) [MIM:601419]; also known as desmin-related myopathy (DRM). CSM is characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by intracytoplasmic accumulation of desmin-reactive deposits in cardiac and skeletal muscle cells. A desmin-related myopathy can have a distal onset, it is then known as hereditary distal myopathy (HDM).,disease:Defects in DES are the cause of neurogenic scapuloperoneal syndrome Kaeser type (Kaeser syndrome) [MIM:181400].
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