Histidine NADH Dehydrogenase Fe-S Protein 4, Recombinant Protein

NDUFS4 Human; Histidine NADH Dehydrogenase Fe-S Protein 4 Human Recombinant; AQDQ; NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4 mitochondrial; NADH-ubiquinone oxidoreductase 18 kDa subunit; Complex I-18 kDa; CI-18 kDa; Complex I-AQDQ; CI-AQDQ

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

E Coli

Greater than 90.0% as determined by SDS-PAGE.

The NDUFS4 solution contains 20mM Tris pH-8 & 30% glycerol.
Sterile Filtered colorless solution.

NDUFS4 although stable 4 degree C for 4 weeks, should be stored desiccated 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.

Small volumes of NDUFS4 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: NDUFS4 Human Recombinant produced in E Coli is a single, non-glycosylated, polypeptide chain containing 134 amino acids (43-175 a.a.) and having a molecular mass of 15.5 kDa.The NDUFS4 is purified by proprietary chromatographic techniques.

Introduction: NDUFS4 is a subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), the primary multi-subunit enzyme complex of the mitochondrial respiratory chain. Complex I is involved in cellular ATP production, the main source of energy for numerous vital processes in living cells. NDUFS4 removes electrons from NADH and passes them by a series of diverse protein-coupled redox centers to the electron acceptor ubiquinone. NDUFS4 presents a hotspot of mutations in the genetic apparatus of oxidative phosphorylation and the correct assembly of the subunit it encodes is essential for completion of the assembly of complex I.

ENZYMES; Enzymes; Dehydrogenase

20,108 Da

NADH dehydrogenase (ubiquinone) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase)

NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial; CI-18 kDa; CI-AQDQ; NADH dehydrogenase (ubiquinone) iron-sulfur protein 4; NADH-coenzyme Q reductase, 18-KD; NADH-ubiquinone oxidoreductase 18 kDa subunit; complex I 18kDa subunit; complex I-18 kDa; complex I-AQDQ; mitochondrial respiratory chain complex I (18-KD subunit)

NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial

CI-18 kDa; CI-AQDQ  [Similar Products]

NDUS4_HUMAN

This gene encodes an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), or NADH:ubiquinone oxidoreductase, the first multi-subunit enzyme complex of the mitochondrial respiratory chain. Complex I plays a vital role in cellular ATP production, the primary source of energy for many crucial processes in living cells. It removes electrons from NADH and passes them by a series of different protein-coupled redox centers to the electron acceptor ubiquinone. In well-coupled mitochondria, the electron flux leads to ATP generation via the building of a proton gradient across the inner membrane. Complex I is composed of at least 41 subunits, of which 7 are encoded by the mitochondrial genome and the remainder by nuclear genes. [provided by RefSeq, Jul 2008]

NDUFS4: Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in 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 NDUFS4 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 NDUFS4 subunit family.

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

Chromosomal Location of Human Ortholog: 5q11.1

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

Molecular Function: NADH dehydrogenase (ubiquinone) activity

Biological Process: cellular metabolic process; mitochondrial respiratory chain complex I assembly; positive regulation of fibroblast proliferation; cAMP-mediated signaling; regulation of protein amino acid phosphorylation; response to cAMP; mitochondrial electron transport, NADH to ubiquinone; cellular respiration; brain development

Disease: Leigh Syndrome; Mitochondrial Complex I Deficiency

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