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Home/Reagents/Antibody/Primary antibody/Monoclonal Antibody/Mouse mAb/Desmin [ABT168] mouse mAb
SKU: WM1414 Categories: ,

Desmin [ABT168] mouse mAb

Ucallm_WM1414-50

Price range: $268.00 through $328.00

SKU: WM1414-50
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Product details

Background:
Desmin is an intermediate silk protein with a molecular weight of 53 kDa, which is widely distributed in smooth muscle cells, skeletal muscle cells, cardiomyocytes and myofibroblasts. It is mainly used for the diagnosis and differential diagnosis of smooth muscle tumors and striated muscle tumors. In rhabdomyosarcoma, pleomorphic rhabdomyosarcoma was strongly positive, while embryonic / acinar rhabdomyosarcoma was low or even negative. Myofibroblastoma was positive, and the fibroproliferative small round cell tumor showed paranuclear punctate staining.

Specifications

Size50μL/100μL
TargetDesmin
ReactivityHuman, Mouse, Rat
ApplicationsIHC, WB, IF, ELISA
MW(Calculated)53kD
MW(Observed)60kD
Host SpeciesMouse
IsotypeIgG2b,Kappa
Conjugate/ModificationUnmodified
Recommended Dilution RatioIHC 1:200-1000; WB 1:500-2000; IF 1:100-500; ELISA 1:1000-5000 / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
FormulationPBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
PurificationRecombinant Antibody expressed in animal component-free (ACF) media, purified via Protein G affinity chromatography.
Storage-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
ClonalityMonoclonal
Clone NumberRMM168
ImmunogenSynthesized peptide derived from human Desmin AA range: 400-470
SpecificityThe antibody can specifically recognize human Desmin protein.
Gene NameDES
Protein NameCMD1I; CSM1; CSM2; DES; DESM_HUMAN; Desmin; FLJ12025; FLJ39719; FLJ41013; FLJ41793; Intermediate filament protein; OTTHUMP00000064865
Other NameCMD1I; / CSM1; / CSM2; / DES; / DESM_HUMAN; / Desmin; / FLJ12025; / FLJ39719; / FLJ41013; / FLJ41793; / Intermediate filament protein; / OTTHUMP00000064865
SpeciesHuman
Gene ID1674
UniprotP17661,
Species.1Mouse
Gene ID.1
Uniprot.1P31001,
Species.2Rat
Gene ID.2
Uniprot.2P48675
Cellular LocalizationCytoplasmic
Tissue ExpressionAppendix/ Colon
Signaling_pathway
Research Areas>>Hypertrophic cardiomyopathy; / >>Arrhythmogenic right ventricular cardiomyopathy; / >>Dilated cardiomyopathy
FunctionDisease: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]. Kaeser syndrome is an autosomal dominant disorder with a peculiar scapuloperoneal distribution of weakness and atrophy. A large clinical variability is observed ranging from scapuloperoneal, limb grindle and distal phenotypes with variable cardiac or respiratory involvement. Facial weakness, dysphagia and gynaecomastia are frequent additional symptoms. Affected men seemingly bear a higher risk of sudden, cardiac death as compared to affected women. Histological and immunohistochemical examination of muscle biopsy specimens reveal a wide spectrum of findings ranging from near normal or unspecific pathology to typical, myofibrillar changes with accumulation of desmin.,Function:Desmin are 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.,online information:Desmin entry,similarity:Belongs to the intermediate filament family.,subunit:Homopolymer.,
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