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Identification
HMDB Protein ID HMDBP02016
Secondary Accession Numbers
  • 7472
Name Molybdopterin synthase catalytic subunit
Synonyms
  1. MOCO1-B
  2. MOCS2B
  3. MPT synthase large subunit
  4. Molybdenum cofactor synthesis protein 2 large subunit
  5. Molybdenum cofactor synthesis protein 2B
  6. Molybdopterin-synthase large subunit
Gene Name MOCS2
Protein Type Enzyme
Biological Properties
General Function Involved in Mo-molybdopterin cofactor biosynthetic process
Specific Function Catalytic subunit of the molybdopterin synthase complex, a complex that catalyzes the conversion of precursor Z into molybdopterin. Acts by mediating the incorporation of 2 sulfur atoms from thiocarboxylated MOCS2A into precursor Z to generate a dithiolene group.
Pathways
  • Folate biosynthesis
  • molybdopterin biosynthesis
  • Sulfur relay system
Reactions
Cyclic pyranopterin monophosphate + [molybdopterin-synthase sulfur-carrier protein]-Gly-NH-CH(2)-C(O)SH + Water → Molybdopterin + [molybdopterin-synthase sulfur-carrier protein] details
Cyclic pyranopterin monophosphate + Sulfur donor → Molybdopterin details
GO Classification
Biological Process
water-soluble vitamin metabolic process
Cellular Component
cytosol
molybdopterin synthase complex
nucleus
Molecular Function
Mo-molybdopterin synthase activity
transferase activity
Process
metabolic process
coenzyme biosynthetic process
cellular metabolic process
cofactor metabolic process
coenzyme metabolic process
mo-molybdopterin cofactor biosynthetic process
Cellular Location
  1. Cytoplasm
  2. cytosol
Gene Properties
Chromosome Location 5
Locus 5q11
SNPs MOCS2
Gene Sequence
>567 bp
ATGTCGAGCTTGGAGATCAGCTCCTCGTGCTTCAGCCTGGAGACGAAATTGCCGTTATCC
CCCCCATTAGTGGAGGATAGTGCTTTTGAGCCATCTAGGAAAGATATGGATGAAGTTGAA
GAGAAATCTAAAGATGTTATAAACTTTACTGCCGAGAAACTTTCAGTAGATGAAGTCTCA
CAGTTGGTGATTTCTCCGCTCTGTGGTGCAATATCCCTATTTGTAGGGACTACAAGAAAT
AACTTTGAAGGGAAAAAAGTCATTAGCTTAGAATATGAAGCATATCTACCCATGGCGGAA
AATGAAGTCAGAAAGATTTGTAGTGACATTAGGCAGAAATGGCCAGTCAAACACATAGCA
GTGTTCCATAGACTTGGCTTGGTTCCAGTGTCAGAAGCAAGCATAATCATTGCTGTGTCC
TCAGCCCACAGAGCTGCATCTCTTGAAGCTGTGAGCTATGCCATTGATACTTTAAAAGCC
AAGGTGCCCATATGGAAAAAGGAAATATACGAAGAGTCATCAACTTGGAAAGGAAACAAA
GAGTGCTTTTGGGCATCCAACAGTTAA
Protein Properties
Number of Residues 188
Molecular Weight 20943.735
Theoretical pI 5.443
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Molybdopterin synthase catalytic subunit
MSSLEISSSCFSLETKLPLSPPLVEDSAFEPSRKDMDEVEEKSKDVINFTAEKLSVDEVS
QLVISPLCGAISLFVGTTRNNFEGKKVISLEYEAYLPMAENEVRKICSDIRQKWPVKHIA
VFHRLGLVPVSEASIIIAVSSAHRAASLEAVSYAIDTLKAKVPIWKKEIYEESSTWKGNK
ECFWASNS
GenBank ID Protein 4262373
UniProtKB/Swiss-Prot ID O96007
UniProtKB/Swiss-Prot Entry Name MOC2B_HUMAN
PDB IDs
GenBank Gene ID AF091871
GeneCard ID MOCS2
GenAtlas ID MOCS2
HGNC ID HGNC:7193
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. 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. [PubMed:18669648 ]
  4. Mayya V, Lundgren DH, Hwang SI, Rezaul K, Wu L, Eng JK, Rodionov V, Han DK: Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions. Sci Signal. 2009 Aug 18;2(84):ra46. doi: 10.1126/scisignal.2000007. [PubMed:19690332 ]
  5. Daub H, Olsen JV, Bairlein M, Gnad F, Oppermann FS, Korner R, Greff Z, Keri G, Stemmann O, Mann M: Kinase-selective enrichment enables quantitative phosphoproteomics of the kinome across the cell cycle. Mol Cell. 2008 Aug 8;31(3):438-48. doi: 10.1016/j.molcel.2008.07.007. [PubMed:18691976 ]
  6. 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. [PubMed:16964243 ]
  7. Stallmeyer B, Drugeon G, Reiss J, Haenni AL, Mendel RR: Human molybdopterin synthase gene: identification of a bicistronic transcript with overlapping reading frames. Am J Hum Genet. 1999 Mar;64(3):698-705. [PubMed:10053003 ]
  8. Sloan J, Kinghorn JR, Unkles SE: The two subunits of human molybdopterin synthase: evidence for a bicistronic messenger RNA with overlapping reading frames. Nucleic Acids Res. 1999 Feb 1;27(3):854-8. [PubMed:9889283 ]
  9. Leimkuhler S, Freuer A, Araujo JA, Rajagopalan KV, Mendel RR: Mechanistic studies of human molybdopterin synthase reaction and characterization of mutants identified in group B patients of molybdenum cofactor deficiency. J Biol Chem. 2003 Jul 11;278(28):26127-34. Epub 2003 May 5. [PubMed:12732628 ]
  10. Matthies A, Rajagopalan KV, Mendel RR, Leimkuhler S: Evidence for the physiological role of a rhodanese-like protein for the biosynthesis of the molybdenum cofactor in humans. Proc Natl Acad Sci U S A. 2004 Apr 20;101(16):5946-51. Epub 2004 Apr 8. [PubMed:15073332 ]
  11. Reiss J, Dorche C, Stallmeyer B, Mendel RR, Cohen N, Zabot MT: Human molybdopterin synthase gene: genomic structure and mutations in molybdenum cofactor deficiency type B. Am J Hum Genet. 1999 Mar;64(3):706-11. [PubMed:10053004 ]
  12. Leimkuhler S, Charcosset M, Latour P, Dorche C, Kleppe S, Scaglia F, Szymczak I, Schupp P, Hahnewald R, Reiss J: Ten novel mutations in the molybdenum cofactor genes MOCS1 and MOCS2 and in vitro characterization of a MOCS2 mutation that abolishes the binding ability of molybdopterin synthase. Hum Genet. 2005 Oct;117(6):565-70. Epub 2005 Jul 14. [PubMed:16021469 ]
  13. Hahnewald R, Leimkuhler S, Vilaseca A, Acquaviva-Bourdain C, Lenz U, Reiss J: A novel MOCS2 mutation reveals coordinated expression of the small and large subunit of molybdopterin synthase. Mol Genet Metab. 2006 Nov;89(3):210-3. Epub 2006 Jun 5. [PubMed:16737835 ]