catalog number :
MBS537523
products type :
Native Protein
products full name :
Desmin protein (Chicken)
products short name :
Desmin
other names :
desmin; Desmin; desmin; desmin
other gene names :
DES; DES; CSM1; CSM2; LGMD2R
uniprot entry name :
DESM_HUMAN
form :
Supplied as a lyophilized powder. Reconstitute with 250 ul of distilled water, final solution contains 10 mM Sodium Phosphat buffer pH 7.6, 6M urea, 2mM DTT, 1mM EDTA, and 20mM NaCl.
storage stability :
Store at 4 degree C until reconstitution. Following reconstitution aliquot and freeze at -20 degree C for long term storage. Avoid repeated freeze-thaw cycles.
tested application :
User optimized
other info1 :
Source Note: Chicken gizzard. Protein Type: Native. Biological Significance: Desmin is the main intermediate filament in mature skeletal, cardiac and smooth-muscle cells. The antibody can be used to distinguish rhabdomyosarcomas and leiomyosarcomas from other soft tissue tumors, lymphomas and carcinomas. Mab D9 does not crossreact with other intermediate filament proteins and is useful for the identification of myodifferentiation (also in tumors).
products categories :
Cell Biology ; Native Protein
products description :
Purified native Chicken Desmin protein
ncbi acc num :
NP_001918.3
ncbi gb acc num :
NM_001927.3
ncbi mol weight :
53,536 Da
ncbi pathways :
Arrhythmogenic Right Ventricular Cardiomyopathy Pathway (672454); Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway (117293); Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway (116129); Aurora B Signaling Pathway (138080); Dilated Cardiomyopathy Pathway (121494); Dilated Cardiomyopathy Pathway (121285); Hypertrophic Cardiomyopathy (HCM) Pathway (114229); Hypertrophic Cardiomyopathy (HCM) Pathway (106591); Muscle Contraction Pathway (106261); Striated Muscle Contraction Pathway (198903)
ncbi summary :
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]
uniprot summary :
desmin: a class-III intermediate filaments found in muscle cells. 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. Mutations in this gene are associated with desmin-related myopathy, a familial cardiac and skeletal myopathy (CSM), and with distal myopathies. Protein type: Cytoskeletal; Motility/polarity/chemotaxis. Chromosomal Location of Human Ortholog: 2q35. Cellular Component: intermediate filament; fascia adherens; Z disc; neuromuscular junction; cytosol; sarcolemma. Molecular Function: identical protein binding; protein binding; structural constituent of cytoskeleton; cytoskeletal protein binding. Biological Process: muscle contraction; cytoskeleton organization and biogenesis; regulation of heart contraction; muscle filament sliding. Disease: Cardiomyopathy, Dilated, 1i; Scapuloperoneal Syndrome, Neurogenic, Kaeser Type; Myopathy, Myofibrillar, 1; Muscular Dystrophy, Limb-girdle, Type 2r