NP_002487.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
23,705 Da
NCBI Official Full Name
NADH dehydrogenase
NCBI Official Synonym Full Names
NADH dehydrogenase (ubiquinone) Fe-S protein 8, 23kDa (NADH-coenzyme Q reductase)
NCBI Protein Information
NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial; complex I-23kD; complex I 23kDa subunit; NADH-ubiquinone oxidoreductase 23 kDa subunit
UniProt Protein Name
NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial
UniProt Synonym Protein Names
Complex I-23kD; CI-23kD; NADH-ubiquinone oxidoreductase 23 kDa subunit; TYKY subunit
UniProt Synonym Gene Names
UniProt Entry Name
NDUS8_HUMAN
NCBI Summary for NDUFS8
This gene encodes a subunit of mitochondrial NADH:ubiquinone oxidoreductase, or Complex I, a multimeric enzyme of the respiratory chain responsible for NADH oxidation, ubiquinone reduction, and the ejection of protons from mitochondria. The encoded protein is involved in the binding of two of the six to eight iron-sulfur clusters of Complex I and, as such, is required in the electron transfer process. Mutations in this gene have been associated with Leigh syndrome. [provided by RefSeq, Mar 2010]
UniProt Comments for NDUFS8
TYKY: 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. May donate electrons to ubiquinone. Defects in NDUFS8 are a cause of Leigh syndrome (LS). LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Belongs to the complex I 23 kDa subunit family.
Protein type: Energy Metabolism - oxidative phosphorylation; Mitochondrial; EC 1.6.5.3; EC 1.6.99.3; Oxidoreductase
Chromosomal Location of Human Ortholog: 11q13
Cellular Component: mitochondrion; mitochondrial inner membrane; mitochondrial respiratory chain complex I
Molecular Function: NADH dehydrogenase (ubiquinone) activity; metal ion binding; 4 iron, 4 sulfur cluster binding; NADH dehydrogenase activity
Biological Process: cellular metabolic process; mitochondrial respiratory chain complex I assembly; mitochondrial electron transport, NADH to ubiquinone; response to oxidative stress
Disease: Leigh Syndrome
Research Articles on NDUFS8
Precautions
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Pathways associated with NDUFS8 elisa kit
Diseases associated with NDUFS8 elisa kit
Organs/Tissues associated with NDUFS8 elisa kit
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