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Identification
HMDB Protein ID HMDBP00140
Secondary Accession Numbers
  • 5372
  • HMDBP03481
Name NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial
Synonyms
  1. CI-30kD
  2. Complex I-30kD
  3. NADH-ubiquinone oxidoreductase 30 kDa subunit
Gene Name NDUFS3
Protein Type Unknown
Biological Properties
General Function Involved in oxidoreductase activity, acting on NADH or NADPH
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
reactive oxygen species metabolic process
induction of apoptosis
negative regulation of cell growth
Cellular Component
mitochondrial respiratory chain complex I
Function
catalytic activity
nadh dehydrogenase activity
nadh dehydrogenase (quinone) activity
nadh dehydrogenase (ubiquinone) activity
oxidoreductase activity
oxidoreductase activity, acting on nadh or nadph
Molecular Function
electron carrier activity
NADH dehydrogenase (ubiquinone) activity
Process
metabolic process
oxidation reduction
Cellular Location
  1. Mitochondrion inner membrane
Gene Properties
Chromosome Location 11
Locus 11p11.11
SNPs NDUFS3
Gene Sequence
>795 bp
ATGGCGGCGGCGGCGGTAGCCAGGCTGTGGTGGCGCGGGATCTTGGGGGCCTCGGCGCTG
ACCAGGGGGACTGGGCGACCCTCCGTTCTGTTGCTGCCGGTGAGGCGGGAGAGCGCCGGG
GCCGACACGCGCCCCACTGTCAGACCACGGAATGATGTGGCCCACAAGCAGCTCTCAGCT
TTTGGAGAGTATGTGGCTGAAATCTTGCCCAAGTATGTCCAACAAGTTCAGGTGTCCTGC
TTCAATGAGTTAGAGGTCTGTATCCATCCTGATGGCGTCATCCCAGTGCTGACTTTCCTC
AGGGATCACACCAATGCACAGTTCAAATCTCTGGTTGACTTGACAGCAGTGGACGTCCCA
ACTCGGCAAAACCGTTTTGAGATTGTCTACAACCTGTTGTCTCTGCGCTTCAACTCACGG
ATCCGTGTGAAGACCTACACAGATGAGCTGACGCCCATTGAGTCTGCTGTCTCTGTGTTC
AAGGCAGCCAACTGGTATGAAAGGGAGATCTGGGACATGTTTGGAGTCTTCTTTGCTAAC
CACCCTGATCTAAGAAGGATCCTGACAGATTATGGCTTCGAGGGACATCCTTTCCGGAAA
GACTTTCCTCTATCTGGCTATGTTGAGTTACGTTATGATGATGAAGTGAAGCGGGTGGTG
GCAGAGCCGGTGGAGTTGGCCCAAGAGTTCCGCAAATTTGACCTGAACAGCCCCTGGGAG
GCTTTCCCAGTCTATCGCCAACCCCCGGAGAGTCTCAAGCTTGAAGCCGGAGACAAGAAG
CCTGATGCCAAGTAG
Protein Properties
Number of Residues 264
Molecular Weight 30241.245
Theoretical pI 7.497
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial
MAAAAVARLWWRGILGASALTRGTGRPSVLLLPVRRESAGADTRPTVRPRNDVAHKQLSA
FGEYVAEILPKYVQQVQVSCFNELEVCIHPDGVIPVLTFLRDHTNAQFKSLVDLTAVDVP
TRQNRFEIVYNLLSLRFNSRIRVKTYTDELTPIESAVSVFKAANWYEREIWDMFGVFFAN
HPDLRRILTDYGFEGHPFRKDFPLSGYVELRYDDEVKRVVAEPVELAQEFRKFDLNSPWE
AFPVYRQPPESLKLEAGDKKPDAK
GenBank ID Protein Not Available
UniProtKB/Swiss-Prot ID O75489
UniProtKB/Swiss-Prot Entry Name NDUS3_HUMAN
PDB IDs Not Available
GenBank Gene ID AF067139
GeneCard ID NDUFS3
GenAtlas ID NDUFS3
HGNC ID HGNC:7710
References
General References
  1. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K, Kimura K, Makita H, Sekine M, Obayashi M, Nishi T, Shibahara T, Tanaka T, Ishii S, Yamamoto J, Saito K, Kawai Y, Isono Y, Nakamura Y, Nagahari K, Murakami K, Yasuda T, Iwayanagi T, Wagatsuma M, Shiratori A, Sudo H, Hosoiri T, Kaku Y, Kodaira H, Kondo H, Sugawara M, Takahashi M, Kanda K, Yokoi T, Furuya T, Kikkawa E, Omura Y, Abe K, Kamihara K, Katsuta N, Sato K, Tanikawa M, Yamazaki M, Ninomiya K, Ishibashi T, Yamashita H, Murakawa K, Fujimori K, Tanai H, Kimata M, Watanabe M, Hiraoka S, Chiba Y, Ishida S, Ono Y, Takiguchi S, Watanabe S, Yosida M, Hotuta T, Kusano J, Kanehori K, Takahashi-Fujii A, Hara H, Tanase TO, Nomura Y, Togiya S, Komai F, Hara R, Takeuchi K, Arita M, Imose N, Musashino K, Yuuki H, Oshima A, Sasaki N, Aotsuka S, Yoshikawa Y, Matsunawa H, Ichihara T, Shiohata N, Sano S, Moriya S, Momiyama H, Satoh N, Takami S, Terashima Y, Suzuki O, Nakagawa S, Senoh A, Mizoguchi H, Goto Y, Shimizu F, Wakebe H, Hishigaki H, Watanabe T, Sugiyama A, Takemoto M, Kawakami B, Yamazaki M, Watanabe K, Kumagai A, Itakura S, Fukuzumi Y, Fujimori Y, Komiyama M, Tashiro H, Tanigami A, Fujiwara T, Ono T, Yamada K, Fujii Y, Ozaki K, Hirao M, Ohmori Y, Kawabata A, Hikiji T, Kobatake N, Inagaki H, Ikema Y, Okamoto S, Okitani R, Kawakami T, Noguchi S, Itoh T, Shigeta K, Senba T, Matsumura K, Nakajima Y, Mizuno T, Morinaga M, Sasaki M, Togashi T, Oyama M, Hata H, Watanabe M, Komatsu T, Mizushima-Sugano J, Satoh T, Shirai Y, Takahashi Y, Nakagawa K, Okumura K, Nagase T, Nomura N, Kikuchi H, Masuho Y, Yamashita R, Nakai K, Yada T, Nakamura Y, Ohara O, Isogai T, Sugano S: Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat Genet. 2004 Jan;36(1):40-5. Epub 2003 Dec 21. [PubMed:14702039 ]
  2. 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 ]
  3. Hu RM, Han ZG, Song HD, Peng YD, Huang QH, Ren SX, Gu YJ, Huang CH, Li YB, Jiang CL, Fu G, Zhang QH, Gu BW, Dai M, Mao YF, Gao GF, Rong R, Ye M, Zhou J, Xu SH, Gu J, Shi JX, Jin WR, Zhang CK, Wu TM, Huang GY, Chen Z, Chen MD, Chen JL: Gene expression profiling in the human hypothalamus-pituitary-adrenal axis and full-length cDNA cloning. Proc Natl Acad Sci U S A. 2000 Aug 15;97(17):9543-8. [PubMed:10931946 ]
  4. 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 ]
  5. Loeffen J, van den Heuvel L, Smeets R, Triepels R, Sengers R, Trijbels F, Smeitink J: cDNA sequence and chromosomal localization of the remaining three human nuclear encoded iron sulphur protein (IP) subunits of complex I: the human IP fraction is completed. Biochem Biophys Res Commun. 1998 Jun 29;247(3):751-8. [PubMed:9647766 ]
  6. Saada A, Vogel RO, Hoefs SJ, van den Brand MA, Wessels HJ, Willems PH, Venselaar H, Shaag A, Barghuti F, Reish O, Shohat M, Huynen MA, Smeitink JA, van den Heuvel LP, Nijtmans LG: Mutations in NDUFAF3 (C3ORF60), encoding an NDUFAF4 (C6ORF66)-interacting complex I assembly protein, cause fatal neonatal mitochondrial disease. Am J Hum Genet. 2009 Jun;84(6):718-27. doi: 10.1016/j.ajhg.2009.04.020. Epub 2009 May 21. [PubMed:19463981 ]
  7. Xu G, Shin SB, Jaffrey SR: Global profiling of protease cleavage sites by chemoselective labeling of protein N-termini. Proc Natl Acad Sci U S A. 2009 Nov 17;106(46):19310-5. doi: 10.1073/pnas.0908958106. Epub 2009 Nov 5. [PubMed:19892738 ]
  8. Procaccio V, Lescuyer P, Bourges I, Beugnot R, Duborjal H, Depetris D, Mousson B, Montfort MF, Smeets H, De Coo R, Issartel JP: Human NDUFS3 gene coding for the 30-kDa subunit of mitochondrial complex I: genomic organization and expression. Mamm Genome. 2000 Sep;11(9):808-10. [PubMed:10967146 ]