| IED ID | IndEnz0002006590 |
| Enzyme Type ID | protease006590 |
| Protein Name |
NADH dehydrogenase ubiquinone iron-sulfur protein 7, mitochondrial EC 7.1.1.2 Complex I-20kD CI-20kD NADH-ubiquinone oxidoreductase 20 kDa subunit PSST subunit |
| Gene Name | NDUFS7 |
| 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 | MAVLSAPGLRGFRILGLRSSVGPAVQARGVHQSVATDGPSSTQPALPKARAVAPKPSSRGEYVVAKLDDLVNWARRSSLWPMTFGLACCAVEMMHMAAPRYDMDRFGVVFRASPRQSDVMIVAGTLTNKMAPALRKVYDQMPEPRYVVSMGSCANGGGYYHYSYSVVRGCDRIVPVDIYIPGCPPTAEALLYGILQLQRKIKRERRLQIWYRR |
| Enzyme Length | 213 |
| Uniprot Accession Number | O75251 |
| 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:17275378}; |
| 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:17275378). Essential for the catalytic activity of complex I (PubMed:17275378). {ECO:0000269|PubMed:17275378}. |
| Temperature Dependency | |
| PH Dependency | |
| Pathway | |
| nucleotide Binding | |
| Features | Alternative sequence (1); Beta strand (2); Chain (1); Erroneous gene model prediction (1); Helix (9); Metal binding (4); Modified residue (1); Natural variant (3); Region (1); Transit peptide (1); Turn (3) |
| Keywords | 3D-structure;4Fe-4S;Alternative splicing;Disease variant;Electron transport;Hydroxylation;Iron;Iron-sulfur;Leigh syndrome;Membrane;Metal-binding;Mitochondrion;Mitochondrion inner membrane;NAD;Oxidoreductase;Primary mitochondrial disease;Reference proteome;Respiratory chain;Transit peptide;Translocase;Transport;Ubiquinone |
| Interact With | Q8WXH2 |
| Induction | |
| Subcellular Location | SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000305|PubMed:12611891}; Peripheral membrane protein {ECO:0000250|UniProtKB:P42026}; Matrix side {ECO:0000250|UniProtKB:P42026}. |
| Modified Residue | MOD_RES 111; /note=Hydroxyarginine; /evidence=ECO:0000250|UniProtKB:P42026 |
| Post Translational Modification | PTM: Hydroxylated ar Arg-111 by NDUFAF5 early in the pathway of assembly of complex I, before the formation of the juncture between peripheral and membrane arms. {ECO:0000269|PubMed:27226634}. |
| Signal Peptide | |
| Structure 3D | Electron microscopy (4) |
| Cross Reference PDB | 5XTB; 5XTD; 5XTH; 5XTI; |
| Mapped Pubmed ID | 11220739; 11695836; 12231006; 12837546; 14741580; 15250827; 16169070; 16230336; 17557076; 17601350; 17604671; 18435906; 18977241; 19688755; 20406883; 20552642; 20877624; 21911578; 21924235; 22935918; 23270816; 24191001; 24344204; 28844695; 9878551; |
| Motif | |
| Gene Encoded By | |
| Mass | 23,564 |
| Kinetics | |
| Metal Binding | METAL 88; /note=Iron-sulfur (4Fe-4S); /evidence=ECO:0000255; METAL 89; /note=Iron-sulfur (4Fe-4S); /evidence=ECO:0000255; METAL 153; /note=Iron-sulfur (4Fe-4S); /evidence=ECO:0000255; METAL 183; /note=Iron-sulfur (4Fe-4S); /evidence=ECO:0000255 |
| Rhea ID | RHEA:29091 |
| Cross Reference Brenda |