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DESM Antibody

Mouse Monoclonal Antibody (Mab)

     
  • 1 - DESM Antibody AM1926b
    DESM Antibody (Cat. #AM1926b) western blot analysis in CEM cell line lysates (35μg/lane).This demonstrates the DESM antibody detected the DESM protein (arrow).
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Product info
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, E
Primary Accession P17661
Other Accession NP_001918.3
Reactivity Human
Host Mouse
Clonality Monoclonal
Isotype IgM,k
Clone Names 290CT7.1.1
Calculated MW 53536 Da
Additional info
Gene ID 1674
Other Names Desmin, DES
Target/Specificity This DESM monoclonal antibody is generated from mouse immunized with DESM recombinant protein.
Dilution WB~~1:100~250
Format Purified monoclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Euglobin precipitation followed by dialysis against PBS.
StorageMaintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
PrecautionsDESM Antibody is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name DES
Function Muscle-specific type III intermediate filament essential for proper muscular structure and function. Plays a crucial role in maintaining the structure of sarcomeres, inter-connecting the Z-disks and forming the myofibrils, linking them not only to the sarcolemmal cytoskeleton, but also to the nucleus and mitochondria, thus providing strength for the muscle fiber during activity (PubMed:25358400). In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z-line structures (PubMed:24200904, PubMed:25394388, PubMed:26724190). May act as a sarcomeric microtubule-anchoring protein: specifically associates with detyrosinated tubulin-alpha chains, leading to buckled microtubules and mechanical resistance to contraction. Contributes to the transcriptional regulation of the NKX2-5 gene in cardiac progenitor cells during a short period of cardiomyogenesis and in cardiac side population stem cells in the adult. Plays a role in maintaining an optimal conformation of nebulette (NEB) on heart muscle sarcomeres to bind and recruit cardiac alpha-actin (By similarity).
Cellular Location Cytoplasm, myofibril, sarcomere, Z line Cytoplasm. Cell membrane, sarcolemma. Nucleus {ECO:0000250|UniProtKB:P31001}. Note=Localizes in the intercalated disks which occur at the Z line of cardiomyocytes (PubMed:24200904, PubMed:26724190). Localizes in the nucleus exclusively in differentiating cardiac progenitor cells and premature cardiomyocytes (By similarity) {ECO:0000250|UniProtKB:P31001, ECO:0000269|PubMed:24200904, ECO:0000269|PubMed:26724190}
Research Areas

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].

REFERENCES

Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
van Spaendonck-Zwarts, K., et al. Clin. Genet. (2010) In press :
Zimmerman, R.S., et al. Genet. Med. 12(5):268-278(2010)
Bar, H., et al. J. Mol. Biol. 397(5):1188-1198(2010)
Levin, J., et al. J. Neuropathol. Exp. Neurol. 69(4):415-424(2010)

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