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Identification
HMDB Protein ID HMDBP00180
Secondary Accession Numbers
  • 5412
  • HMDBP03485
Name NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial
Synonyms
  1. CI-20kD
  2. Complex I-20kD
  3. NADH-ubiquinone oxidoreductase 20 kDa subunit
  4. PSST subunit
Gene Name NDUFS7
Protein Type Unknown
Biological Properties
General Function Involved in NADH dehydrogenase (ubiquinone) activity
Specific Function 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 (By similarity).
Pathways
  • Alzheimer's disease
  • Doxorubicin Metabolism Pathway
  • Huntington's disease
  • Oxidative phosphorylation
  • Parkinson's disease
Reactions
NADH + Coenzyme Q10 → NAD + QH(2) details
NADH + acceptor → NAD + reduced acceptor details
GO Classification
Biological Process
small molecule metabolic process
mitochondrial electron transport, NADH to ubiquinone
mitochondrial respiratory chain complex I assembly
Cellular Component
mitochondrial respiratory chain complex I
Function
binding
catalytic activity
nadh dehydrogenase activity
quinone binding
nadh dehydrogenase (quinone) activity
nadh dehydrogenase (ubiquinone) activity
metal cluster binding
iron-sulfur cluster binding
4 iron, 4 sulfur cluster binding
cofactor binding
oxidoreductase activity
oxidoreductase activity, acting on nadh or nadph
Molecular Function
metal ion binding
4 iron, 4 sulfur cluster binding
NADH dehydrogenase (ubiquinone) activity
quinone binding
Process
metabolic process
oxidation reduction
Cellular Location
  1. Mitochondrion
Gene Properties
Chromosome Location 19
Locus 19p13.3
SNPs NDUFS7
Gene Sequence
>642 bp
ATGGCGGTGCTGTCAGCTCCTGGCCTGCGCGGCTTCCGGATCCTTGGTCTGCGCTCCAGC
GTGGGCCCGGCTGTGCAGGCACGAGGTGTCCATCAGAGCGTGGCCACCGATGGCCCAAGC
AGCACCCAGCCTGCCCTGCCAAAGGCCAGAGCCGTGGCTCCCAAACCCAGCAGCCGGGGC
GAGTATGTGGTGGCCAAGCTGGATGACCTCGTCAACTGGGCCCGCCGGAGTTCTCTGTGG
CCCATGACCTTCGGCCTGGCCTGCTGCGCCGTGGAGATGATGCACATGGCAGCACCCCGC
TACGACATGGACCGCTTTGGCGTGGTCTTCCGCGCCAGCCCGCGCCAGTCCGACGTCATG
ATCGTGGCCGGCACACTCACCAACAAGATGGCCCCAGCGCTTCGCAAGGTCTACGACCAG
ATGCCGGAGCCGCGCTACGTGGTCTCCATGGGGAGCTGCGCCAACGGAGGAGGCTACTAC
CACTATTCCTACTCGGTGGTGAGGGGCTGCGACCGCATCGTGCCCGTGGACATCTACATC
CCAGGCTGCCCACCTACGGCCGAGGCCCTGCTCTACGGCATCCTGCAGCTGCAGAGGAAG
ATCAAGCGGGAGCGGAGGCTGCAGATCTGGTACCGCAGGTAG
Protein Properties
Number of Residues 213
Molecular Weight 23563.3
Theoretical pI 9.995
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial
MAVLSAPGLRGFRILGLRSSVGPAVQARGVHQSVATDGPSSTQPALPKARAVAPKPSSRG
EYVVAKLDDLVNWARRSSLWPMTFGLACCAVEMMHMAAPRYDMDRFGVVFRASPRQSDVM
IVAGTLTNKMAPALRKVYDQMPEPRYVVSMGSCANGGGYYHYSYSVVRGCDRIVPVDIYI
PGCPPTAEALLYGILQLQRKIKRERRLQIWYRR
GenBank ID Protein 187281616
UniProtKB/Swiss-Prot ID O75251
UniProtKB/Swiss-Prot Entry Name NDUS7_HUMAN
PDB IDs Not Available
GenBank Gene ID NM_024407.4
GeneCard ID NDUFS7
GenAtlas ID NDUFS7
HGNC ID HGNC:7714
References
General References
  1. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
  2. Grimwood J, Gordon LA, Olsen A, Terry A, Schmutz J, Lamerdin J, Hellsten U, Goodstein D, Couronne O, Tran-Gyamfi M, Aerts A, Altherr M, Ashworth L, Bajorek E, Black S, Branscomb E, Caenepeel S, Carrano A, Caoile C, Chan YM, Christensen M, Cleland CA, Copeland A, Dalin E, Dehal P, Denys M, Detter JC, Escobar J, Flowers D, Fotopulos D, Garcia C, Georgescu AM, Glavina T, Gomez M, Gonzales E, Groza M, Hammon N, Hawkins T, Haydu L, Ho I, Huang W, Israni S, Jett J, Kadner K, Kimball H, Kobayashi A, Larionov V, Leem SH, Lopez F, Lou Y, Lowry S, Malfatti S, Martinez D, McCready P, Medina C, Morgan J, Nelson K, Nolan M, Ovcharenko I, Pitluck S, Pollard M, Popkie AP, Predki P, Quan G, Ramirez L, Rash S, Retterer J, Rodriguez A, Rogers S, Salamov A, Salazar A, She X, Smith D, Slezak T, Solovyev V, Thayer N, Tice H, Tsai M, Ustaszewska A, Vo N, Wagner M, Wheeler J, Wu K, Xie G, Yang J, Dubchak I, Furey TS, DeJong P, Dickson M, Gordon D, Eichler EE, Pennacchio LA, Richardson P, Stubbs L, Rokhsar DS, Myers RM, Rubin EM, Lucas SM: The DNA sequence and biology of human chromosome 19. Nature. 2004 Apr 1;428(6982):529-35. [PubMed:15057824 ]
  3. Murray J, Zhang B, Taylor SW, Oglesbee D, Fahy E, Marusich MF, Ghosh SS, Capaldi RA: The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification. J Biol Chem. 2003 Apr 18;278(16):13619-22. Epub 2003 Feb 28. [PubMed:12611891 ]
  4. Hyslop SJ, Duncan AM, Pitkanen S, Robinson BH: Assignment of the PSST subunit gene of human mitochondrial complex I to chromosome 19p13. Genomics. 1996 Nov 1;37(3):375-80. [PubMed:8938450 ]
  5. Triepels RH, van den Heuvel LP, Loeffen JL, Buskens CA, Smeets RJ, Rubio Gozalbo ME, Budde SM, Mariman EC, Wijburg FA, Barth PG, Trijbels JM, Smeitink JA: Leigh syndrome associated with a mutation in the NDUFS7 (PSST) nuclear encoded subunit of complex I. Ann Neurol. 1999 Jun;45(6):787-90. [PubMed:10360771 ]
  6. Smeitink J, van den Heuvel L: Human mitochondrial complex I in health and disease. Am J Hum Genet. 1999 Jun;64(6):1505-10. [PubMed:10330338 ]