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O96007

- MOC2B_HUMAN

UniProt

O96007 - MOC2B_HUMAN

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Protein

Molybdopterin synthase catalytic subunit

Gene

MOCS2

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli

Functioni

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.2 PublicationsUniRule annotation

Catalytic activityi

Cyclic pyranopterin phosphate + 2 [molybdopterin-synthase sulfur-carrier protein]-Gly-NH-CH(2)-C(O)SH + H2O = molybdopterin + 2 [molybdopterin-synthase sulfur-carrier protein].2 PublicationsUniRule annotation

Pathwayi

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei159 – 1591SubstrateUniRule annotation

GO - Molecular functioni

  1. molybdopterin synthase activity Source: UniProtKB

GO - Biological processi

  1. molybdopterin cofactor biosynthetic process Source: Reactome
  2. Mo-molybdopterin cofactor biosynthetic process Source: UniProtKB
  3. small molecule metabolic process Source: Reactome
  4. vitamin metabolic process Source: Reactome
  5. water-soluble vitamin metabolic process Source: Reactome
Complete GO annotation...

Keywords - Molecular functioni

Transferase

Keywords - Biological processi

Molybdenum cofactor biosynthesis

Enzyme and pathway databases

BioCyciMetaCyc:HS09033-MONOMER.
ReactomeiREACT_25073. Molybdenum cofactor biosynthesis.
UniPathwayiUPA00344.

Names & Taxonomyi

Protein namesi
Recommended name:
Molybdopterin synthase catalytic subunitUniRule annotation (EC:2.8.1.12UniRule annotation)
Alternative name(s):
MOCO1-B
Molybdenum cofactor synthesis protein 2 large subunitUniRule annotation
Molybdenum cofactor synthesis protein 2BUniRule annotation
Short name:
MOCS2BUniRule annotation
Molybdopterin-synthase large subunit
Short name:
MPT synthase large subunit
Gene namesi
Name:MOCS2UniRule annotation
Synonyms:MCBPE, MOCO1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640: Chromosome 5

Organism-specific databases

HGNCiHGNC:7193. MOCS2.

Subcellular locationi

Cytoplasmcytosol 1 PublicationUniRule annotation

GO - Cellular componenti

  1. cytoplasm Source: HPA
  2. cytosol Source: UniProtKB
  3. molybdopterin synthase complex Source: UniProtKB
  4. nucleus Source: HPA
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm

Pathology & Biotechi

Involvement in diseasei

Molybdenum cofactor deficiency, complementation group B (MOCODB) [MIM:252160]: An autosomal recessive metabolic disorder characterized by neonatal onset of intractable seizures, opisthotonus, and facial dysmorphism associated with hypouricemia and elevated urinary sulfite levels. Affected individuals show severe neurologic damage and often die in early childhood.3 Publications
Note: The disease is caused by mutations affecting the gene represented in this entry.
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti168 – 1681E → K in MOCODB. 1 Publication
VAR_012765

Keywords - Diseasei

Disease mutation

Organism-specific databases

MIMi252160. phenotype.
PharmGKBiPA30903.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 188188Molybdopterin synthase catalytic subunitPRO_0000163111Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei20 – 201Phosphoserine5 Publications

Keywords - PTMi

Phosphoprotein

Proteomic databases

MaxQBiO96007.
PRIDEiO96007.

PTM databases

PhosphoSiteiO96007.

Expressioni

Tissue specificityi

Highest levels are found in heart and skeletal muscle. Lower levels are present in brain, kidney and pancreas. Very low levels are found in lung and peripheral blood leukocytes.1 Publication

Gene expression databases

BgeeiO96007.
CleanExiHS_MOCS2.
ExpressionAtlasiO96007. baseline and differential.
GenevestigatoriO96007.

Organism-specific databases

HPAiHPA037680.

Interactioni

Subunit structurei

Heterotetramer; composed of 2 small (MOCS2A) and 2 large (MOCS2B) subunits.UniRule annotation

Protein-protein interaction databases

BioGridi110481. 3 interactions.
IntActiO96007. 3 interactions.
STRINGi9606.ENSP00000339580.

Structurei

Secondary structure

1
188
Legend: HelixTurnBeta strand
Show more details
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Beta strandi45 – 528
Helixi56 – 627
Beta strandi69 – 779
Beta strandi80 – 823
Beta strandi85 – 9612
Helixi98 – 11316
Beta strandi116 – 1249
Beta strandi126 – 1294
Beta strandi132 – 14312
Helixi144 – 16118
Beta strandi164 – 1707

3D structure databases

Select the link destinations:
PDBe
RCSB PDB
PDBj
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
4AP8X-ray2.78A/B/C/D38-172[»]
ProteinModelPortaliO96007.
SMRiO96007. Positions 38-171.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni143 – 1442Substrate bindingUniRule annotation
Regioni166 – 1683Substrate bindingUniRule annotation

Sequence similaritiesi

Belongs to the MoaE family. MOCS2B subfamily.UniRule annotation

Phylogenomic databases

GeneTreeiENSGT00510000047669.
HOGENOMiHOG000280991.
InParanoidiO96007.
KOiK03635.
OMAiGNKECFW.
PhylomeDBiO96007.
TreeFamiTF314334.

Family and domain databases

Gene3Di3.90.1170.40. 1 hit.
HAMAPiMF_03052. MOC2B.
InterProiIPR028888. MOCS2B_euk.
IPR003448. Mopterin_biosynth_MoaE.
[Graphical view]
PANTHERiPTHR23404:SF2. PTHR23404:SF2. 1 hit.
PfamiPF02391. MoaE. 1 hit.
[Graphical view]
SUPFAMiSSF54690. SSF54690. 1 hit.

Sequencei

Sequence statusi: Complete.

O96007-1 [UniParc]FASTAAdd to Basket

« Hide

        10         20         30         40         50
MSSLEISSSC FSLETKLPLS PPLVEDSAFE PSRKDMDEVE EKSKDVINFT
60 70 80 90 100
AEKLSVDEVS QLVISPLCGA ISLFVGTTRN NFEGKKVISL EYEAYLPMAE
110 120 130 140 150
NEVRKICSDI RQKWPVKHIA VFHRLGLVPV SEASIIIAVS SAHRAASLEA
160 170 180
VSYAIDTLKA KVPIWKKEIY EESSTWKGNK ECFWASNS
Length:188
Mass (Da):20,944
Last modified:May 1, 1999 - v1
Checksum:iF405256D85621146
GO

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti50 – 501T → A.
Corresponds to variant rs2233213 [ dbSNP | Ensembl ].
VAR_050091
Natural varianti77 – 771T → A.
Corresponds to variant rs2233215 [ dbSNP | Ensembl ].
VAR_050092
Natural varianti123 – 1231H → Y.
Corresponds to variant rs2233218 [ dbSNP | Ensembl ].
VAR_050093
Natural varianti168 – 1681E → K in MOCODB. 1 Publication
VAR_012765
Natural varianti187 – 1871N → S.
Corresponds to variant rs2233221 [ dbSNP | Ensembl ].
VAR_050094

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
AF091871 mRNA. Translation: AAD14599.1.
AF117815 mRNA. Translation: AAD13297.1.
AF155659 mRNA. Translation: AAF67478.1.
AK312887 mRNA. Translation: BAG35735.1.
CR457172 mRNA. Translation: CAG33453.1.
CH471123 Genomic DNA. Translation: EAW54874.1.
BC046097 mRNA. Translation: AAH46097.1.
CCDSiCCDS3958.1.
PIRiB59370.
RefSeqiNP_004522.1. NM_004531.4.
UniGeneiHs.163645.
Hs.594335.

Genome annotation databases

EnsembliENST00000396954; ENSP00000380157; ENSG00000164172.
GeneIDi4338.
KEGGihsa:4338.
UCSCiuc003joz.3. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
AF091871 mRNA. Translation: AAD14599.1 .
AF117815 mRNA. Translation: AAD13297.1 .
AF155659 mRNA. Translation: AAF67478.1 .
AK312887 mRNA. Translation: BAG35735.1 .
CR457172 mRNA. Translation: CAG33453.1 .
CH471123 Genomic DNA. Translation: EAW54874.1 .
BC046097 mRNA. Translation: AAH46097.1 .
CCDSi CCDS3958.1.
PIRi B59370.
RefSeqi NP_004522.1. NM_004531.4.
UniGenei Hs.163645.
Hs.594335.

3D structure databases

Select the link destinations:
PDBe
RCSB PDB
PDBj
Links Updated
Entry Method Resolution (Å) Chain Positions PDBsum
4AP8 X-ray 2.78 A/B/C/D 38-172 [» ]
ProteinModelPortali O96007.
SMRi O96007. Positions 38-171.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

BioGridi 110481. 3 interactions.
IntActi O96007. 3 interactions.
STRINGi 9606.ENSP00000339580.

PTM databases

PhosphoSitei O96007.

Proteomic databases

MaxQBi O96007.
PRIDEi O96007.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENST00000396954 ; ENSP00000380157 ; ENSG00000164172 .
GeneIDi 4338.
KEGGi hsa:4338.
UCSCi uc003joz.3. human.

Organism-specific databases

CTDi 4338.
GeneCardsi GC05M052429.
HGNCi HGNC:7193. MOCS2.
HPAi HPA037680.
MIMi 252160. phenotype.
603708. gene.
neXtProti NX_O96007.
PharmGKBi PA30903.
GenAtlasi Search...

Phylogenomic databases

GeneTreei ENSGT00510000047669.
HOGENOMi HOG000280991.
InParanoidi O96007.
KOi K03635.
OMAi GNKECFW.
PhylomeDBi O96007.
TreeFami TF314334.

Enzyme and pathway databases

UniPathwayi UPA00344 .
BioCyci MetaCyc:HS09033-MONOMER.
Reactomei REACT_25073. Molybdenum cofactor biosynthesis.

Miscellaneous databases

GeneWikii MOCS2.
GenomeRNAii 4338.
NextBioi 17072.
PROi O96007.
SOURCEi Search...

Gene expression databases

Bgeei O96007.
CleanExi HS_MOCS2.
ExpressionAtlasi O96007. baseline and differential.
Genevestigatori O96007.

Family and domain databases

Gene3Di 3.90.1170.40. 1 hit.
HAMAPi MF_03052. MOC2B.
InterProi IPR028888. MOCS2B_euk.
IPR003448. Mopterin_biosynth_MoaE.
[Graphical view ]
PANTHERi PTHR23404:SF2. PTHR23404:SF2. 1 hit.
Pfami PF02391. MoaE. 1 hit.
[Graphical view ]
SUPFAMi SSF54690. SSF54690. 1 hit.
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "Human molybdopterin synthase gene: identification of a bicistronic transcript with overlapping reading frames."
    Stallmeyer B., Drugeon G., Reiss J., Haenni A.L., Mendel R.R.
    Am. J. Hum. Genet. 64:698-705(1999) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA], IDENTIFICATION OF BICISTRONIC GENE, TISSUE SPECIFICITY.
    Tissue: Liver.
  2. "The two subunits of human molybdopterin synthase: evidence for a bicistronic messenger RNA with overlapping reading frames."
    Sloan J., Kinghorn J.R., Unkles S.E.
    Nucleic Acids Res. 27:854-858(1999) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
  3. Huang C., Huang Q., Wu T., Peng Y., Gu Y., Zhang L., Jiang C., Li Y., Han Z., Wang Y., Chen Z., Fu G.
    Submitted (JUN-1999) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Tissue: Adrenal gland.
  4. "Complete sequencing and characterization of 21,243 full-length human cDNAs."
    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 T.-O., 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.
    Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Uterus.
  5. "Cloning of human full open reading frames in Gateway(TM) system entry vector (pDONR201)."
    Ebert L., Schick M., Neubert P., Schatten R., Henze S., Korn B.
    Submitted (JUN-2004) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
  6. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  7. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Duodenum.
  8. "Mechanistic studies of human molybdopterin synthase reaction and characterization of mutants identified in group B patients of molybdenum cofactor deficiency."
    Leimkuehler S., Freuer A., Araujo J.A., Rajagopalan K.V., Mendel R.R.
    J. Biol. Chem. 278:26127-26134(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: ENZYME ACTIVITY, FUNCTION, SUBUNIT.
  9. "Evidence for the physiological role of a rhodanese-like protein for the biosynthesis of the molybdenum cofactor in humans."
    Matthies A., Rajagopalan K.V., Mendel R.R., Leimkuehler S.
    Proc. Natl. Acad. Sci. U.S.A. 101:5946-5951(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: ENZYME ACTIVITY, FUNCTION, SUBUNIT, SUBCELLULAR LOCATION.
  10. "A probability-based approach for high-throughput protein phosphorylation analysis and site localization."
    Beausoleil S.A., Villen J., Gerber S.A., Rush J., Gygi S.P.
    Nat. Biotechnol. 24:1285-1292(2006) [PubMed] [Europe PMC] [Abstract]
    Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-20, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Cervix carcinoma.
  11. "Kinase-selective enrichment enables quantitative phosphoproteomics of the kinome across the cell cycle."
    Daub H., Olsen J.V., Bairlein M., Gnad F., Oppermann F.S., Korner R., Greff Z., Keri G., Stemmann O., Mann M.
    Mol. Cell 31:438-448(2008) [PubMed] [Europe PMC] [Abstract]
    Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-20, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Cervix carcinoma.
  12. Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-20, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Cervix carcinoma.
  13. "Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions."
    Mayya V., Lundgren D.H., Hwang S.-I., Rezaul K., Wu L., Eng J.K., Rodionov V., Han D.K.
    Sci. Signal. 2:RA46-RA46(2009) [PubMed] [Europe PMC] [Abstract]
    Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-20, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Leukemic T-cell.
  14. "Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis."
    Olsen J.V., Vermeulen M., Santamaria A., Kumar C., Miller M.L., Jensen L.J., Gnad F., Cox J., Jensen T.S., Nigg E.A., Brunak S., Mann M.
    Sci. Signal. 3:RA3-RA3(2010) [PubMed] [Europe PMC] [Abstract]
    Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-20, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Cervix carcinoma.
  15. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  16. "Human molybdopterin synthase gene: genomic structure and mutations in molybdenum cofactor deficiency type B."
    Reiss J., Dorche C., Stallmeyer B., Mendel R.R., Cohen N., Zabot M.-T.
    Am. J. Hum. Genet. 64:706-711(1999) [PubMed] [Europe PMC] [Abstract]
    Cited for: VARIANT MOCODB LYS-168.
  17. "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."
    Leimkuehler S., Charcosset M., Latour P., Dorche C., Kleppe S., Scaglia F., Szymczak I., Schupp P., Hahnewald R., Reiss J.
    Hum. Genet. 117:565-570(2005) [PubMed] [Europe PMC] [Abstract]
    Cited for: INVOLVEMENT IN MOCODB.
  18. "A novel MOCS2 mutation reveals coordinated expression of the small and large subunit of molybdopterin synthase."
    Hahnewald R., Leimkuehler S., Vilaseca A., Acquaviva-Bourdain C., Lenz U., Reiss J.
    Mol. Genet. Metab. 89:210-213(2006) [PubMed] [Europe PMC] [Abstract]
    Cited for: INVOLVEMENT IN MOCODB.

Entry informationi

Entry nameiMOC2B_HUMAN
AccessioniPrimary (citable) accession number: O96007
Secondary accession number(s): Q6IAI3
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 11, 2002
Last sequence update: May 1, 1999
Last modified: October 29, 2014
This is version 122 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Miscellaneous

This protein is produced by a bicistronic gene which also produces the small subunit (MOCS2A) from an overlapping reading frame. Expression of these 2 proteins are related since a mutation that removes the start codon of the small subunit (MOCS2A) also impairs expression of the large subunit (MOCS2B).

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 5
    Human chromosome 5: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3