Desmin (ABT19R) rabbit mAb

  • Catalog No.：YM7110
  • Applications：IHC;WB; ELISA
  • Reactivity：Human;Mouse;(predicted: Rat)
    • Target：
    • Desmin
    • Fields：
    • >>Hypertrophic cardiomyopathy;>>Arrhythmogenic right ventricular cardiomyopathy;>>Dilated cardiomyopathy
    • Gene Name：
    • DES
    • Protein Name：
    • Desmin
    • Human Gene Id：
    • 1674
    • Human Swiss Prot No：
    • P17661
    • Immunogen：
    • Synthesized peptide derived from human Desmin AA range:400-470
    • Specificity：
    • This antibody detects endogenous levels of Desmin
    • Formulation：
    • PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
    • Source：
    • Monoclonal, Rabbit IgG1, Kappa
    • Dilution：
    • IHC 1:100-500, WB 1:500-1000, ELISA 1:5000-20000
    • Purification：
    • Recombinant Expression and Affinity purified
    • Storage Stability：
    • -15°C to -25°C/1 year(Do not lower than -25°C)
    • Other Name：
    • CMD1I;CSM1;CSM2;DES;DESM_HUMAN;Desmin;FLJ12025;FLJ39719;FLJ41013;FLJ41793;Intermediate filament protein;OTTHUMP00000064865
    • Molecular Weight(Da)：
    • 54kD
    • 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].
    • Subcellular Location：
    • Cytoplasmic
    • Expression：
    • Cytoplasmic

    Attributes

    • Code：
    • YM7110
    • Size
    • 40μL / 100μL / 200μL
    • Price$：
    • 190 / 340 / 600
    • Delivery：
    • 2024-07-24
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