protein

Recombinant Human Desmin

MBS143072

0.005 mg

140 USD

Recombinant Protein

Recombinant Human Desmin

Desmin

Desmin Human; Desmin Human Recombinant; Desmin; DES; CSM1; CSM2; CMD1I; FLJ12025; FLJ39719; FLJ41013; FLJ41793; Desmin

desmin; Desmin; desmin; intermediate filament protein; mutant desmin p.K241E; desmin

DES; DES; CSM1; CSM2; LGMD2R

DESM_HUMAN

E Coli

470

Greater than 95.0% as determined by: (a) Analysis by RP-HPLC. (b) Analysis by SDS-PAGE.

Desmin was lyophilized from a 1mg/ml solution containing 30mM Tris-HCl pH 8, 9.5M urea, 2mM DTT, 2mM EDTA and 10mM methylammonium chloride. Sterile Filtered White lyophilized (freeze-dried) powder.

Lyophilized Desmin although stable at room temperature for 3 weeks, should be stored desiccated below -18 degree C. Upon reconstitution Desmin should be stored at 4 degree C between 2-7 days and for future use below -18 degree C.For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Please prevent freeze-thaw cycles.

Protein standard in 1D and 2D SDS gelelectrophoresisImmunoassays Immunization.

Solubility: It is recommended to reconstitute the lyophilized Desmin in sterile 18M Omega -cm H2O not less than 100 ug/ml, which can then be further diluted to other aqueous solutions.

RECOMBINANT & NATURAL PROTEINS; Recombinant Proteins

Description: Desmin Human Recombinant having a Molecular mass of 53,539 Dalton. Introduction: Desmin is 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.

55749932

NP_001918.3

NM_001927.3

P17661

53,536 Da

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)

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]

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: Motility/polarity/chemotaxis; Cytoskeletal. Chromosomal Location of Human Ortholog: 2q35. Cellular Component: intermediate filament; fascia adherens; cytosol; neuromuscular junction; Z disc; 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

0.005 mg

140 USD

0.02 mg

205

1 mg

3490