Human NDUFS2 Recombinant Protein (RPPB2011)
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주문- SKU:
- RPPB2011
- Product type:
- Recombinant Protein
- Size:
- 10ug
- Species:
- Human
- Target:
- NDUFS2
- Synonyms:
- CI-49
- Synonyms:
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 2
- Synonyms:
- mitochondrial
- Synonyms:
- Complex I-49kD
- Source:
- Escherichia Coli
- Uniprot:
- O75306
Description
| Product Name: | Human NDUFS2 Recombinant Protein |
| Product Code: | RPPB2011 |
| Size: | 10µg |
| Species: | Human |
| Target: | NDUFS2 |
| Synonyms: | CI-49 , NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial, Complex I-49kD, CI-49kD, NADH-ubiquinone oxidoreductase 49 kDa subunit, NDUFS2. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered clear solution. |
| Formulation: | The NDUFS2 solution (0.25mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 0.4M Urea and 10% glycerol. |
| Stability: | Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles. |
| Purity: | Greater than 80% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGSSHHHHHH SSGLVPRGSH MGSVKNITLN FGPQHPAAHG VLRLVMELSG EMVRKCDPHI GLLHRGTEKL IEYKTYLQAL PYFDRLDYVS MMCNEQAYSL AVEKLLNIRP PPRAQWIRVL FGEITRLLNH IMAVTTHALD LGAMTPFFWL FEEREKMFEF YERVSGARMH AAYIRPGGVH QDLPLGLMDD IYQFSKNFSL RLDELEELLT NNRIWRNRTI DIGVVTAEEA LNYGFSGVML RGSGIQWDLR KTQPYDVYDQ VEFDVPVGSR GDCYDRYLCR VEEMRQSLRI IAQCLNKMPP GEIKVDDAKV SPPKRAEMKT SMESLIHHFK LYTEGYQVPP GATYTAIEAP KGEFGVYLVS DGSSRPYRCK IKAPGFAHLA GLDKMSKGHM LADVVAIIGT QDIVFGEVDR |
NDUFS2 is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which is a part of the minimal assembly required for catalysis. Complex I takes part in the transfer of electrons from NADH to the respiratory chain. Histidine NADH Dehydrogenase Fe-S Protein 2 (NDUFS2) is required for catalytic activity. Imperfections in NDUFS2 are the source of complex I mitochondrial respiratory chain deficiency, which is characterized by many symptoms including liver failure, cardiomyopathy and neurodegeneration.
NDUFS2 Human Recombinant produced in E.coli is a single, non-glycosylated polypeptide chain containing 410 amino acids (77-463a.a) and having a molecular mass of 46.5kDa. NDUFS2 is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.
| UniProt Protein Function: | NDUFS2: Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. Defects in NDUFS2 are a cause of mitochondrial complex I deficiency (MT-C1D). A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. Belongs to the complex I 49 kDa subunit family. |
| UniProt Protein Details: | Protein type:Oxidoreductase; Mitochondrial; EC 1.6.5.3; EC 1.6.99.3; Energy Metabolism - oxidative phosphorylation Chromosomal Location of Human Ortholog: 1q23 Cellular Component: mitochondrial matrix; mitochondrial respiratory chain complex I; mitochondrion; nucleoplasm Molecular Function:NADH dehydrogenase activity; protein binding; ubiquitin protein ligase binding Biological Process: mitochondrial electron transport, NADH to ubiquinone; mitochondrial respiratory chain complex I assembly; response to oxidative stress Disease: Mitochondrial Complex I Deficiency |
| NCBI Summary: | The protein encoded by this gene is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I). Mammalian mitochondrial complex I is composed of at least 43 different subunits, 7 of which are encoded by the mitochondrial genome, and the rest are the products of nuclear genes. The iron-sulfur protein fraction of complex I is made up of 7 subunits, including this gene product. Complex I catalyzes the NADH oxidation with concomitant ubiquinone reduction and proton ejection out of the mitochondria. Mutations in this gene are associated with mitochondrial complex I deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Oct 2009] |
| UniProt Code: | O75306 |
| NCBI GenInfo Identifier: | 20178314 |
| NCBI Gene ID: | 4720 |
| NCBI Accession: | O75306.2 |
| UniProt Secondary Accession: | O75306,Q5VTW0, Q969P3, Q9UEV3, D3DVG7, J3KPM7, |
| UniProt Related Accession: | O75306 |
| Molecular Weight: | 51,852 Da |
| NCBI Full Name: | NADH dehydrogenase |
| NCBI Synonym Full Names: | NADH:ubiquinone oxidoreductase core subunit S2 |
| NCBI Official Symbol: | NDUFS2 |
| NCBI Official Synonym Symbols: | CI-49 |
| NCBI Protein Information: | NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial |
| UniProt Protein Name: | NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial |
| UniProt Synonym Protein Names: | Complex I-49kD; CI-49kD; NADH-ubiquinone oxidoreductase 49 kDa subunit |
| UniProt Gene Name: | NDUFS2 |
| UniProt Entry Name: | NDUS2_HUMAN |
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