| IED ID | IndEnz0002012368 |
| Enzyme Type ID | protease012368 |
| Protein Name |
NADH dehydrogenase ubiquinone iron-sulfur protein 3, mitochondrial EC 7.1.1.2 Complex I-30kD CI-30kD NADH-ubiquinone oxidoreductase 30 kDa subunit |
| Gene Name | NDUFS3 |
| Organism | Homo sapiens (Human) |
| Taxonomic Lineage | cellular organisms Eukaryota Opisthokonta Metazoa Eumetazoa Bilateria Deuterostomia Chordata Craniata Vertebrata Gnathostomata (jawed vertebrates) Teleostomi Euteleostomi Sarcopterygii Dipnotetrapodomorpha Tetrapoda Amniota Mammalia Theria Eutheria Boreoeutheria Euarchontoglires Primates Haplorrhini Simiiformes Catarrhini Hominoidea (apes) Hominidae (great apes) Homininae Homo Homo sapiens (Human) |
| Enzyme Sequence | MAAAAVARLWWRGILGASALTRGTGRPSVLLLPVRRESAGADTRPTVRPRNDVAHKQLSAFGEYVAEILPKYVQQVQVSCFNELEVCIHPDGVIPVLTFLRDHTNAQFKSLVDLTAVDVPTRQNRFEIVYNLLSLRFNSRIRVKTYTDELTPIESAVSVFKAANWYEREIWDMFGVFFANHPDLRRILTDYGFEGHPFRKDFPLSGYVELRYDDEVKRVVAEPVELAQEFRKFDLNSPWEAFPVYRQPPESLKLEAGDKKPDAK |
| Enzyme Length | 264 |
| Uniprot Accession Number | O75489 |
| Absorption | |
| Active Site | |
| Activity Regulation | |
| Binding Site | |
| Calcium Binding | |
| catalytic Activity | CATALYTIC ACTIVITY: Reaction=a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) + NAD(+); Xref=Rhea:RHEA:29091, Rhea:RHEA-COMP:9565, Rhea:RHEA-COMP:9566, ChEBI:CHEBI:15378, ChEBI:CHEBI:16389, ChEBI:CHEBI:17976, ChEBI:CHEBI:57540, ChEBI:CHEBI:57945; EC=7.1.1.2; Evidence={ECO:0000269|PubMed:14729820, ECO:0000269|PubMed:30140060}; |
| DNA Binding | |
| EC Number | 7.1.1.2 |
| Enzyme Function | FUNCTION: Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor (PubMed:14729820, PubMed:30140060). Essential for the catalytic activity and assembly of complex I (PubMed:14729820, PubMed:24028823, PubMed:30140060). {ECO:0000269|PubMed:14729820, ECO:0000269|PubMed:24028823, ECO:0000269|PubMed:30140060}. |
| Temperature Dependency | |
| PH Dependency | |
| Pathway | |
| nucleotide Binding | |
| Features | Alternative sequence (2); Beta strand (11); Chain (1); Helix (5); Natural variant (4); Sequence conflict (1); Transit peptide (1); Turn (4) |
| Keywords | 3D-structure;Alternative splicing;Direct protein sequencing;Disease variant;Electron transport;Membrane;Mitochondrion;Mitochondrion inner membrane;NAD;Oxidoreductase;Primary mitochondrial disease;Reference proteome;Respiratory chain;Transit peptide;Translocase;Transport;Ubiquinone |
| Interact With | Q66PJ3-4; Q5JUW0-3; Q9H8H3; Q16718; P51970; O75306; P17152 |
| Induction | |
| Subcellular Location | SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000269|PubMed:18826940, ECO:0000305|PubMed:12611891, ECO:0000305|PubMed:17209039}; Peripheral membrane protein {ECO:0000305|PubMed:18826940}; Matrix side {ECO:0000269|PubMed:18826940}. |
| Modified Residue | |
| Post Translational Modification | |
| Signal Peptide | |
| Structure 3D | Electron microscopy (4) |
| Cross Reference PDB | 5XTB; 5XTD; 5XTH; 5XTI; |
| Mapped Pubmed ID | 11220739; 11695836; 11771736; 12231006; 12376551; 12837546; 14741580; 15250827; 16230336; 16339170; 17027967; 17314511; 17500595; 17557076; 17601350; 18485875; 18977241; 19034380; 19103604; 19343046; 19688755; 19738201; 20064372; 20186120; 20406883; 20552642; 20679435; 20877624; 21310150; 21867691; 21911578; 21924235; 21988832; 22593156; 22623428; 22734669; 23270816; 23446378; 23455922; 23630608; 24191001; 24344204; 24659802; 25609649; 25770168; 26496610; 26638075; 28242297; 28844695; 29142257; 29849492; 31871319; 33097395; 9878551; |
| Motif | |
| Gene Encoded By | |
| Mass | 30,242 |
| Kinetics | |
| Metal Binding | |
| Rhea ID | RHEA:29091 |
| Cross Reference Brenda |