Histidine NADH Dehydrogenase Fe-S Protein 2, Recombinant Protein

NDUFS2 Human; Histidine NADH Dehydrogenase Fe-S Protein 2 Human Recombinant; CI-49; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2; mitochondrial; Complex I-49kD; CI-49kD; NADH-ubiquinone oxidoreductase 49 kDa subunit; NDUFS2

For Research Use Only. Not for use in diagnostic procedures.

E Coli

Greater than 80% as determined by SDS-PAGE.

The NDUFS2 solution (0.25mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 0.4M Urea and 10% glycerol.
Sterile Filtered clear solution.

Small volumes of NDUFS2 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Description: 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.

Introduction: 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.  

ENZYMES; Enzymes; Dehydrogenase

51,852 Da

NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49kDa (NADH-coenzyme Q reductase)

NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial; CI-49kD; NADH-ubiquinone oxidoreductase 49 kDa subunit; NADH-ubiquinone oxidoreductase NDUFS2 subunit; complex 1, mitochondrial respiratory chain, 49-KD subunit; complex I 49kDa subunit; complex I-49kD

NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial

CI-49kD  [Similar Products]

NDUS2_HUMAN

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]

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 *****-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.

Protein type: Mitochondrial; EC 1.6.5.3; Energy Metabolism - oxidative phosphorylation; Oxidoreductase; EC 1.6.99.3

Chromosomal Location of Human Ortholog: 1q23

Cellular Component: nucleoplasm; mitochondrion; mitochondrial inner membrane; mitochondrial respiratory chain complex I

Molecular Function: protein binding; electron carrier activity; NADH dehydrogenase (ubiquinone) activity; metal ion binding; 4 iron, 4 sulfur cluster binding; NAD binding; quinone binding; NADH dehydrogenase activity

Biological Process: cellular metabolic process; mitochondrial electron transport, NADH to ubiquinone; response to oxidative stress

Disease: Mitochondrial Complex I Deficiency

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