Description
| Product Name: | Human Desmin Recombinant Protein |
| Product Code: | RPPB3349 |
| Size: | 20µg |
| Species: | Human |
| Target: | Desmin |
| Synonyms: | Desmin, DES, CSM1, CSM2, CMD1I, FLJ12025, FLJ39719, FLJ41013, FLJ41793. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered White lyophilized (freeze-dried) powder. |
| Formulation: | 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. |
| Solubility: | It is recommended to reconstitute the lyophilized Desmin in sterile 18M?-cm H2O not less than 100�g/ml, which can then be further diluted to other aqueous solutions. |
| Stability: | Lyophilized Desmin although stable at room temperature for 3 weeks, should be stored desiccated below -18°C. Upon reconstitution Desmin should be stored at 4°C between 2-7 days and for future use below -18°C.For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Please prevent freeze-thaw cycles. |
| Purity: | Greater than 95.0% as determined by SDS-PAGE. |
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.
Desmin Human Recombinant having a calculated molecular mass of 53,539 Dalton, showing a 55kDa band on SDS-page, pI-5.16.
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | 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 |
| 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 Code: | P17661 |
| NCBI GenInfo Identifier: | 6686280 |
| NCBI Gene ID: | 1674 |
| NCBI Accession: | P17661.3 |
| UniProt Secondary Accession: | P17661,Q15787, Q549R7, Q549R8, Q549R9, Q8IZR1, Q8IZR6 Q8NES2, Q8NEU6, Q8TAC4, Q8TCX2, Q8TD99, |
| UniProt Related Accession: | P17661 |
| Molecular Weight: | |
| NCBI Full Name: | Desmin |
| NCBI Synonym Full Names: | desmin |
| NCBI Official Symbol: | DES�� |
| NCBI Official Synonym Symbols: | CSM1; CSM2; LGMD2R�� |
| NCBI Protein Information: | desmin; mutant desmin p.K241E; intermediate filament protein |
| UniProt Protein Name: | Desmin |
| Protein Family: | Desmin |
| UniProt Gene Name: | DES�� |
| UniProt Entry Name: | DESM_HUMAN |
