Molybdenum cofactor sulfurase

Details

Name
Molybdenum cofactor sulfurase
Synonyms
  • 2.8.1.9
  • MCS
  • Molybdenum cofactor sulfurtransferase
Gene Name
MOCOS
Organism
Humans
Amino acid sequence
>lcl|BSEQ0019246|Molybdenum cofactor sulfurase
MAGAAAESGRELWTFAGSRDPSAPRLAYGYGPGSLRELRAREFSRLAGTVYLDHAGATLF
SQSQLESFTSDLMENTYGNPHSQNISSKLTHDTVEQVRYRILAHFHTTAEDYTVIFTAGS
TAALKLVAEAFPWVSQGPESSGSRFCYLTDSHTSVVGMRNVTMAINVISTPVRPEDLWSA
EERSASASNPDCQLPHLFCYPAQSNFSGVRYPLSWIEEVKSGRLHPVSTPGKWFVLLDAA
SYVSTSPLDLSAHQADFVPISFYKIFGFPTGLGALLVHNRAAPLLRKTYFGGGTASAYLA
GEDFYIPRQSVAQRFEDGTISFLDVIALKHGFDTLERLTGGMENIKQHTFTLAQYTYVAL
SSLQYPNGAPVVRIYSDSEFSSPEVQGPIINFNVLDDKGNIIGYSQVDKMASLYNIHLRT
GCFCNTGACQRHLGISNEMVRKHFQAGHVCGDNMDLIDGQPTGSVRISFGYMSTLDDVQA
FLRFIIDTRLHSSGDWPVPQAHADTGETGAPSADSQADVIPAVMGRRSLSPQEDALTGSR
VWNNSSTVNAVPVAPPVCDVARTQPTPSEKAAGVLEGALGPHVVTNLYLYPIKSCAAFEV
TRWPVGNQGLLYDRSWMVVNHNGVCLSQKQEPRLCLIQPFIDLRQRIMVIKAKGMEPIEV
PLEENSERTQIRQSRVCADRVSTYDCGEKISSWLSTFFGRPCHLIKQSSNSQRNAKKKHG
KDQLPGTMATLSLVNEAQYLLINTSSILELHRQLNTSDENGKEELFSLKDLSLRFRANII
INGKRAFEEEKWDEISIGSLRFQVLGPCHRCQMICIDQQTGQRNQHVFQKLSESRETKVN
FGMYLMHASLDLSSPCFLSVGSQVLPVLKENVEGHDLPASEKHQDVTS
Number of residues
888
Molecular Weight
98118.965
Theoretical pI
6.72
GO Classification
Functions
lyase activity / Mo-molybdopterin cofactor sulfurase activity / molybdenum ion binding / pyridoxal phosphate binding / transferase activity
Processes
Mo-molybdopterin cofactor biosynthetic process / molybdopterin cofactor biosynthetic process / molybdopterin cofactor metabolic process / small molecule metabolic process / vitamin metabolic process / water-soluble vitamin metabolic process
Components
cytosol
General Function
Transferase activity
Specific Function
Sulfurates the molybdenum cofactor. Sulfation of molybdenum is essential for xanthine dehydrogenase (XDH) and aldehyde oxidase (ADO) enzymes in which molybdenum cofactor is liganded by 1 oxygen and 1 sulfur atom in active form. In vitro, the C-terminal domain is able to reduce N-hydroxylated prodrugs, such as benzamidoxime.
Pfam Domain Function
Transmembrane Regions
Not Available
Cellular Location
Not Available
Gene sequence
>lcl|BSEQ0019247|Molybdenum cofactor sulfurase (MOCOS)
ATGGCCGGCGCGGCGGCGGAGTCAGGGCGGGAGCTGTGGACCTTCGCGGGTTCCCGGGAC
CCGAGCGCACCGCGGCTAGCCTACGGCTACGGCCCGGGCAGCCTGCGCGAGCTGCGGGCG
CGCGAGTTCAGCCGCCTGGCAGGAACTGTCTATCTTGACCATGCAGGTGCCACCTTGTTC
TCCCAGAGCCAGCTCGAAAGCTTCACTAGTGATCTCATGGAAAACACTTATGGTAATCCT
CACAGCCAGAACATCAGCAGCAAGCTCACCCATGACACTGTGGAGCAGGTGCGCTACAGA
ATCCTGGCGCACTTCCACACCACCGCAGAAGACTACACTGTGATCTTCACTGCCGGGAGC
ACGGCTGCTCTCAAACTGGTGGCAGAGGCCTTTCCATGGGTGTCCCAGGGCCCAGAGAGC
AGTGGGAGTCGCTTCTGTTACCTCACCGACAGCCACACCTCCGTAGTGGGTATGCGGAAC
GTGACCATGGCTATAAATGTCATATCCACCCCGGTCAGGCCAGAGGACCTGTGGTCTGCA
GAGGAACGTAGTGCTTCAGCCAGCAACCCAGACTGCCAGCTGCCGCATCTCTTCTGCTAC
CCAGCTCAGAGTAACTTTTCTGGAGTCAGATACCCCCTGTCCTGGATAGAAGAGGTCAAG
TCTGGGCGGTTGCACCCTGTGAGCACGCCTGGGAAGTGGTTTGTGCTGCTGGATGCAGCC
TCCTACGTGAGCACCTCGCCTTTGGACCTGTCAGCTCACCAGGCCGACTTTGTCCCCATC
TCCTTCTATAAGATCTTCGGGTTTCCTACAGGCCTGGGCGCTCTGCTGGTCCATAATCGT
GCGGCTCCTCTACTGAGGAAGACCTACTTTGGAGGAGGGACAGCCTCTGCGTACCTAGCA
GGAGAAGACTTCTACATCCCGAGGCAGTCGGTAGCTCAGAGGTTTGAAGATGGCACCATC
TCATTCCTTGATGTTATCGCGCTAAAACATGGATTTGACACCCTAGAGCGCCTCACAGGT
GGAATGGAGAATATAAAGCAGCACACCTTCACCTTGGCTCAGTATACCTACGTGGCCCTG
TCCTCTCTCCAGTACCCCAATGGAGCCCCTGTGGTGCGGATTTACAGCGATTCTGAGTTC
AGCAGCCCTGAGGTTCAGGGCCCAATCATCAATTTTAATGTGCTGGATGACAAAGGGAAC
ATCATTGGTTACTCCCAGGTGGACAAAATGGCCAGTCTTTACAACATCCACCTGCGAACT
GGCTGCTTCTGTAACACTGGGGCCTGCCAGAGGCACCTGGGCATAAGCAACGAGATGGTC
AGGAAGCATTTTCAGGCTGGTCATGTCTGTGGGGACAATATGGACCTCATAGATGGGCAG
CCCACAGGATCTGTGAGGATTTCATTTGGATACATGTCGACGCTGGATGATGTCCAGGCC
TTTCTTAGGTTCATCATAGACACTCGCCTGCACTCATCAGGGGACTGGCCTGTCCCTCAG
GCCCATGCTGACACCGGGGAGACTGGAGCCCCATCAGCAGACAGCCAGGCTGATGTTATA
CCTGCTGTCATGGGCAGACGTAGCCTCTCGCCTCAGGAAGATGCCCTCACAGGCTCCAGG
GTTTGGAACAACTCGTCTACTGTGAATGCTGTGCCTGTGGCCCCACCTGTGTGTGATGTC
GCCAGAACCCAGCCGACTCCTTCAGAGAAAGCTGCAGGAGTCCTGGAGGGGGCCCTTGGG
CCACATGTTGTCACTAACCTTTATCTCTATCCAATCAAATCCTGTGCTGCATTTGAGGTG
ACCAGGTGGCCTGTAGGAAACCAAGGGCTGCTATATGACCGGAGCTGGATGGTTGTGAAT
CACAATGGTGTTTGCCTGAGTCAGAAGCAGGAACCCCGGCTCTGCCTGATCCAGCCCTTC
ATCGACTTGCGGCAAAGGATCATGGTCATCAAAGCCAAAGGGATGGAGCCTATAGAGGTG
CCTCTTGAGGAAAATAGTGAACGGACTCAGATTCGCCAAAGCAGGGTCTGTGCTGACAGA
GTAAGTACTTATGATTGTGGAGAAAAAATTTCAAGCTGGTTGTCAACATTTTTTGGCCGT
CCTTGTCATTTGATCAAACAAAGTTCAAACTCTCAAAGGAATGCAAAGAAGAAACATGGA
AAAGATCAACTTCCTGGTACAATGGCCACCCTTTCTCTGGTGAATGAGGCACAGTATCTG
CTGATCAACACATCCAGTATTTTGGAACTTCACCGGCAACTAAACACCAGTGATGAGAAT
GGAAAGGAGGAATTATTCTCACTGAAGGATCTCAGCTTGCGTTTTCGTGCCAATATTATT
ATCAATGGAAAAAGGGCTTTTGAAGAAGAGAAATGGGATGAGATTTCAATTGGCTCTTTG
CGTTTCCAGGTTTTGGGGCCTTGTCACAGATGCCAGATGATTTGCATCGACCAGCAAACT
GGGCAACGAAACCAGCATGTTTTCCAAAAACTTTCTGAGAGTCGTGAAACAAAGGTGAAC
TTTGGCATGTACCTGATGCATGCATCATTGGATTTATCCTCCCCATGTTTCCTGTCTGTA
GGATCTCAGGTGCTCCCTGTGTTGAAAGAGAATGTGGAAGGTCATGATTTACCTGCATCT
GAGAAACACCAGGATGTTACCTCCTAA
Chromosome Location
18
Locus
18q12
External Identifiers
ResourceLink
UniProtKB IDQ96EN8
UniProtKB Entry NameMOCOS_HUMAN
GenBank Protein ID7021017
GenBank Gene IDAK000740
GenAtlas IDMOCOS
HGNC IDHGNC:18234
General References
  1. Ichida K, Matsumura T, Sakuma R, Hosoya T, Nishino T: Mutation of human molybdenum cofactor sulfurase gene is responsible for classical xanthinuria type II. Biochem Biophys Res Commun. 2001 Apr 20;282(5):1194-200. [Article]
  2. 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. [Article]
  3. Nusbaum C, Zody MC, Borowsky ML, Kamal M, Kodira CD, Taylor TD, Whittaker CA, Chang JL, Cuomo CA, Dewar K, FitzGerald MG, Yang X, Abouelleil A, Allen NR, Anderson S, Bloom T, Bugalter B, Butler J, Cook A, DeCaprio D, Engels R, Garber M, Gnirke A, Hafez N, Hall JL, Norman CH, Itoh T, Jaffe DB, Kuroki Y, Lehoczky J, Lui A, Macdonald P, Mauceli E, Mikkelsen TS, Naylor JW, Nicol R, Nguyen C, Noguchi H, O'Leary SB, O'Neill K, Piqani B, Smith CL, Talamas JA, Topham K, Totoki Y, Toyoda A, Wain HM, Young SK, Zeng Q, Zimmer AR, Fujiyama A, Hattori M, Birren BW, Sakaki Y, Lander ES: DNA sequence and analysis of human chromosome 18. Nature. 2005 Sep 22;437(7058):551-5. [Article]
  4. 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. [Article]
  5. Bechtel S, Rosenfelder H, Duda A, Schmidt CP, Ernst U, Wellenreuther R, Mehrle A, Schuster C, Bahr A, Blocker H, Heubner D, Hoerlein A, Michel G, Wedler H, Kohrer K, Ottenwalder B, Poustka A, Wiemann S, Schupp I: The full-ORF clone resource of the German cDNA Consortium. BMC Genomics. 2007 Oct 31;8:399. [Article]
  6. Havemeyer A, Bittner F, Wollers S, Mendel R, Kunze T, Clement B: Identification of the missing component in the mitochondrial benzamidoxime prodrug-converting system as a novel molybdenum enzyme. J Biol Chem. 2006 Nov 17;281(46):34796-802. Epub 2006 Sep 13. [Article]
  7. Beausoleil SA, Villen J, Gerber SA, Rush J, Gygi SP: A probability-based approach for high-throughput protein phosphorylation analysis and site localization. Nat Biotechnol. 2006 Oct;24(10):1285-92. Epub 2006 Sep 10. [Article]
  8. Dephoure N, Zhou C, Villen J, Beausoleil SA, Bakalarski CE, Elledge SJ, Gygi SP: A quantitative atlas of mitotic phosphorylation. Proc Natl Acad Sci U S A. 2008 Aug 5;105(31):10762-7. doi: 10.1073/pnas.0805139105. Epub 2008 Jul 31. [Article]
  9. Olsen JV, Vermeulen M, Santamaria A, Kumar C, Miller ML, Jensen LJ, Gnad F, Cox J, Jensen TS, Nigg EA, Brunak S, Mann M: Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis. Sci Signal. 2010 Jan 12;3(104):ra3. doi: 10.1126/scisignal.2000475. [Article]
  10. Bian Y, Song C, Cheng K, Dong M, Wang F, Huang J, Sun D, Wang L, Ye M, Zou H: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J Proteomics. 2014 Jan 16;96:253-62. doi: 10.1016/j.jprot.2013.11.014. Epub 2013 Nov 22. [Article]
  11. Yamamoto T, Moriwaki Y, Takahashi S, Tsutsumi Z, Tuneyoshi K, Matsui K, Cheng J, Hada T: Identification of a new point mutation in the human molybdenum cofactor sulferase gene that is responsible for xanthinuria type II. Metabolism. 2003 Nov;52(11):1501-4. [Article]
  12. Peretz H, Naamati MS, Levartovsky D, Lagziel A, Shani E, Horn I, Shalev H, Landau D: Identification and characterization of the first mutation (Arg776Cys) in the C-terminal domain of the Human Molybdenum Cofactor Sulfurase (HMCS) associated with type II classical xanthinuria. Mol Genet Metab. 2007 May;91(1):23-9. Epub 2007 Mar 23. [Article]

Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB00114Pyridoxal phosphateapproved, investigational, nutraceuticalunknowncofactorDetails