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Protein

Molybdopterin synthase catalytic subunit

Gene

MOCS2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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.UniRule annotation2 Publications

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).

Catalytic activityi

Cyclic pyranopterin phosphate + 2 [molybdopterin-synthase sulfur-carrier protein]-Gly-NH-CH2-C(O)SH + H2O = molybdopterin + 2 [molybdopterin-synthase sulfur-carrier protein].UniRule annotation2 Publications

Pathwayi: molybdopterin biosynthesis

This protein is involved in the pathway molybdopterin biosynthesis, which is part of Cofactor biosynthesis.UniRule annotation
View all proteins of this organism that are known to be involved in the pathway molybdopterin biosynthesis and in Cofactor biosynthesis.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei159SubstrateUniRule annotation1

GO - Molecular functioni

  • molybdopterin synthase activity Source: UniProtKB

GO - Biological processi

Keywordsi

Molecular functionTransferase
Biological processMolybdenum cofactor biosynthesis

Enzyme and pathway databases

BioCyciMetaCyc:HS09033-MONOMER
BRENDAi2.8.1.12 2681
ReactomeiR-HSA-947581 Molybdenum cofactor biosynthesis
UniPathwayi
UPA00344

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
Proteomesi
  • UP000005640 Componenti: Chromosome 5

Organism-specific databases

EuPathDBiHostDB:ENSG00000164172.18
HGNCiHGNC:7193 MOCS2
MIMi603708 gene
neXtProtiNX_O96007

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm

Pathology & Biotechi

Involvement in diseasei

Molybdenum cofactor deficiency, complementation group B (MOCODB)3 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn 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.
See also OMIM:252160
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_012765168E → K in MOCODB. 1 PublicationCorresponds to variant dbSNP:rs121908605EnsemblClinVar.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi4338
MalaCardsiMOCS2
MIMi252160 phenotype
OpenTargetsiENSG00000164172
Orphaneti308393 Sulfite oxidase deficiency due to molybdenum cofactor deficiency type B
PharmGKBiPA30903

Polymorphism and mutation databases

BioMutaiMOCS2

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001631111 – 188Molybdopterin synthase catalytic subunitAdd BLAST188

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei20PhosphoserineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiO96007
MaxQBiO96007
PaxDbiO96007
PeptideAtlasiO96007
PRIDEiO96007
ProteomicsDBi51186

PTM databases

iPTMnetiO96007
PhosphoSitePlusiO96007

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

BgeeiENSG00000164172 Expressed in 219 organ(s), highest expression level in caudate nucleus
CleanExiHS_MOCS2
ExpressionAtlasiO96007 baseline and differential
GenevisibleiO96007 HS

Organism-specific databases

HPAiHPA037679
HPA037680

Interactioni

Subunit structurei

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

Protein-protein interaction databases

BioGridi110481, 5 interactors
CORUMiO96007
IntActiO96007, 5 interactors
STRINGi9606.ENSP00000380157

Structurei

Secondary structure

1188
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliO96007
SMRiO96007
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni143 – 144Substrate bindingUniRule annotation2
Regioni166 – 168Substrate bindingUniRule annotation3

Sequence similaritiesi

Belongs to the MoaE family. MOCS2B subfamily.UniRule annotation

Phylogenomic databases

eggNOGiKOG3307 Eukaryota
COG0314 LUCA
GeneTreeiENSGT00510000047669
HOGENOMiHOG000280991
InParanoidiO96007
KOiK03635
OMAiWKKEMYE
OrthoDBiEOG091G0CV5
PhylomeDBiO96007
TreeFamiTF314334

Family and domain databases

CDDicd00756 MoaE, 1 hit
Gene3Di3.90.1170.40, 1 hit
HAMAPiMF_03052 MOC2B, 1 hit
InterProiView protein in InterPro
IPR036563 MoaE_sf
IPR028888 MOCS2B_euk
IPR003448 Mopterin_biosynth_MoaE
PfamiView protein in Pfam
PF02391 MoaE, 1 hit
SUPFAMiSSF54690 SSF54690, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

This entry has 1 described isoform and 2 potential isoforms that are computationally mapped.Show allAlign All

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

Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
O96033MOC2A_HUMAN
Molybdopterin synthase sulfur carri...
MOCS2 MOCO1
88Annotation score:
E9PKT9E9PKT9_HUMAN
Molybdopterin synthase sulfur carri...
MOCS2
83Annotation score:

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_05009150T → A. Corresponds to variant dbSNP:rs2233213Ensembl.1
Natural variantiVAR_05009277T → A. Corresponds to variant dbSNP:rs2233215Ensembl.1
Natural variantiVAR_050093123H → Y. Corresponds to variant dbSNP:rs2233218EnsemblClinVar.1
Natural variantiVAR_012765168E → K in MOCODB. 1 PublicationCorresponds to variant dbSNP:rs121908605EnsemblClinVar.1
Natural variantiVAR_050094187N → S. Corresponds to variant dbSNP:rs2233221Ensembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
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.5 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
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

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
4AP8X-ray2.78A/B/C/D38-172[»]
5MPOX-ray2.43C/D27-179[»]
ProteinModelPortaliO96007
SMRiO96007
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110481, 5 interactors
CORUMiO96007
IntActiO96007, 5 interactors
STRINGi9606.ENSP00000380157

PTM databases

iPTMnetiO96007
PhosphoSitePlusiO96007

Polymorphism and mutation databases

BioMutaiMOCS2

Proteomic databases

EPDiO96007
MaxQBiO96007
PaxDbiO96007
PeptideAtlasiO96007
PRIDEiO96007
ProteomicsDBi51186

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000396954; ENSP00000380157; ENSG00000164172
GeneIDi4338
KEGGihsa:4338
UCSCiuc003joz.5 human

Organism-specific databases

CTDi4338
DisGeNETi4338
EuPathDBiHostDB:ENSG00000164172.18
GeneCardsiMOCS2
HGNCiHGNC:7193 MOCS2
HPAiHPA037679
HPA037680
MalaCardsiMOCS2
MIMi252160 phenotype
603708 gene
neXtProtiNX_O96007
OpenTargetsiENSG00000164172
Orphaneti308393 Sulfite oxidase deficiency due to molybdenum cofactor deficiency type B
PharmGKBiPA30903
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3307 Eukaryota
COG0314 LUCA
GeneTreeiENSGT00510000047669
HOGENOMiHOG000280991
InParanoidiO96007
KOiK03635
OMAiWKKEMYE
OrthoDBiEOG091G0CV5
PhylomeDBiO96007
TreeFamiTF314334

Enzyme and pathway databases

UniPathwayi
UPA00344

BioCyciMetaCyc:HS09033-MONOMER
BRENDAi2.8.1.12 2681
ReactomeiR-HSA-947581 Molybdenum cofactor biosynthesis

Miscellaneous databases

ChiTaRSiMOCS2 human
GeneWikiiMOCS2
GenomeRNAii4338
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000164172 Expressed in 219 organ(s), highest expression level in caudate nucleus
CleanExiHS_MOCS2
ExpressionAtlasiO96007 baseline and differential
GenevisibleiO96007 HS

Family and domain databases

CDDicd00756 MoaE, 1 hit
Gene3Di3.90.1170.40, 1 hit
HAMAPiMF_03052 MOC2B, 1 hit
InterProiView protein in InterPro
IPR036563 MoaE_sf
IPR028888 MOCS2B_euk
IPR003448 Mopterin_biosynth_MoaE
PfamiView protein in Pfam
PF02391 MoaE, 1 hit
SUPFAMiSSF54690 SSF54690, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiMOC2B_HUMAN
AccessioniPrimary (citable) accession number: O96007
Secondary accession number(s): Q6IAI3
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 11, 2002
Last sequence update: May 1, 1999
Last modified: November 7, 2018
This is version 148 of the entry and version 1 of the sequence. See 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

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  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. Human chromosome 5
    Human chromosome 5: entries, gene names and cross-references to MIM
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