NP_004542.1
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NCBI GenBank Nucleotide #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
14,679 Da
NCBI Official Full Name
NADH dehydrogenase
NCBI Official Synonym Full Names
NADH:ubiquinone oxidoreductase core subunit S3
NCBI Official Synonym Symbols
NCBI Protein Information
NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial
UniProt Protein Name
NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial
UniProt Synonym Protein Names
Complex I-30kD; CI-30kD; NADH-ubiquinone oxidoreductase 30 kDa subunit
UniProt Synonym Gene Names
UniProt Entry Name
NDUS3_HUMAN
NCBI Summary for NDUFS3
This gene encodes one of the iron-sulfur protein (IP) components of mitochondrial NADH:ubiquinone oxidoreductase (complex I). Mutations in this gene are associated with Leigh syndrome resulting from mitochondrial complex I deficiency.[provided by RefSeq, Apr 2009]
UniProt Comments for NDUFS3
NDUFS3: 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. Belongs to the complex I 30 kDa subunit family.
Protein type: Mitochondrial; Energy Metabolism - oxidative phosphorylation; EC 1.6.5.3; EC 1.6.99.3; Oxidoreductase
Chromosomal Location of Human Ortholog: 11p11.11
Cellular Component: mitochondrial matrix; mitochondrial membrane; mitochondrial respiratory chain complex I; mitochondrion; myelin sheath; nucleus
Molecular Function: electron carrier activity; NADH dehydrogenase (ubiquinone) activity; NADH dehydrogenase activity; protein binding
Biological Process: mitochondrial electron transport, NADH to ubiquinone; mitochondrial respiratory chain complex I assembly; negative regulation of cell growth; substantia nigra development
Disease: Leigh Syndrome; Mitochondrial Complex I Deficiency
Research Articles on NDUFS3
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Products associated with NDUFS3 blocking peptide
Pathways associated with NDUFS3 blocking peptide
Diseases associated with NDUFS3 blocking peptide
Organs/Tissues associated with NDUFS3 blocking peptide
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