Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Molybdenum cofactor sulfurase

Gene

MOCOS

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

Functioni

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.UniRule annotation1 Publication

Catalytic activityi

Molybdenum cofactor + L-cysteine + reduced acceptor + 2 H+ = thio-molybdenum cofactor + L-alanine + H2O + oxidized acceptor.UniRule annotation

Cofactori

pyridoxal 5'-phosphateUniRule annotation

Kineticsi

  1. KM=15 µM for benzamidoxime1 Publication
  1. Vmax=0.24 nmol/min/mg enzyme1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei424UniRule annotation1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionTransferase
Biological processMolybdenum cofactor biosynthesis
LigandPyridoxal phosphate

Enzyme and pathway databases

ReactomeiR-HSA-947581 Molybdenum cofactor biosynthesis

Names & Taxonomyi

Protein namesi
Recommended name:
Molybdenum cofactor sulfuraseUniRule annotation (EC:2.8.1.9UniRule annotation)
Short name:
MCSUniRule annotation
Short name:
MOSUniRule annotation
Short name:
MoCo sulfuraseUniRule annotation
Short name:
hMCS
Alternative name(s):
Molybdenum cofactor sulfurtransferaseUniRule annotation
Gene namesi
Name:MOCOSUniRule annotation
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 18

Organism-specific databases

EuPathDBiHostDB:ENSG00000075643.5
HGNCiHGNC:18234 MOCOS
MIMi613274 gene
neXtProtiNX_Q96EN8

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

Pathology & Biotechi

Involvement in diseasei

Xanthinuria 2 (XAN2)4 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA disorder characterized by excretion of very large amounts of xanthine in the urine and a tendency to form xanthine stones. Uric acid is strikingly diminished in serum and urine. In addition, XAN2 patients cannot metabolize allopurinol into oxypurinol due to dual deficiency of xanthine dehydrogenase and aldehyde oxidase.
See also OMIM:603592
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_02752857A → P in XAN2. 1 PublicationCorresponds to variant dbSNP:rs886037854EnsemblClinVar.1
Natural variantiVAR_027533294T → I in XAN2. 1 PublicationCorresponds to variant dbSNP:rs577279030Ensembl.1
Natural variantiVAR_045899776R → C in XAN2. 1 PublicationCorresponds to variant dbSNP:rs750896617EnsemblClinVar.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi55034
MalaCardsiMOCOS
MIMi603592 phenotype
OpenTargetsiENSG00000075643
Orphaneti93602 Xanthinuria type II
PharmGKBiPA134964534

Chemistry databases

DrugBankiDB00114 Pyridoxal Phosphate

Polymorphism and mutation databases

BioMutaiMOCOS
DMDMi296438294

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002499521 – 888Molybdenum cofactor sulfuraseAdd BLAST888

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei34PhosphoserineCombined sources1
Modified residuei264N6-(pyridoxal phosphate)lysineUniRule annotation1
Modified residuei528PhosphoserineCombined sources1
Modified residuei530PhosphoserineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiQ96EN8
MaxQBiQ96EN8
PaxDbiQ96EN8
PeptideAtlasiQ96EN8
PRIDEiQ96EN8
ProteomicsDBi76426

PTM databases

iPTMnetiQ96EN8
PhosphoSitePlusiQ96EN8

Expressioni

Gene expression databases

BgeeiENSG00000075643
CleanExiHS_MOCOS
GenevisibleiQ96EN8 HS

Organism-specific databases

HPAiHPA039412
HPA047958

Interactioni

Binary interactionsi

WithEntry#Exp.IntActNotes
PARVAQ9NVD74EBI-1220583,EBI-747655

Protein-protein interaction databases

BioGridi120363, 27 interactors
IntActiQ96EN8, 6 interactors
MINTiQ96EN8
STRINGi9606.ENSP00000261326

Structurei

3D structure databases

ProteinModelPortaliQ96EN8
SMRiQ96EN8
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini706 – 867MOSCUniRule annotationAdd BLAST162

Sequence similaritiesi

Belongs to the class-V pyridoxal-phosphate-dependent aminotransferase family. MOCOS subfamily.UniRule annotation

Phylogenomic databases

eggNOGiKOG2142 Eukaryota
COG0520 LUCA
COG3217 LUCA
GeneTreeiENSGT00530000063150
HOGENOMiHOG000029698
HOVERGENiHBG081980
InParanoidiQ96EN8
KOiK15631
OMAiRVNTYDC
OrthoDBiEOG091G03KL
PhylomeDBiQ96EN8
TreeFamiTF105761

Family and domain databases

Gene3Di3.40.640.10, 1 hit
3.90.1150.10, 1 hit
HAMAPiMF_03050 MOCOS, 1 hit
InterProiView protein in InterPro
IPR000192 Aminotrans_V_dom
IPR005302 MoCF_Sase_C
IPR028886 MoCo_sulfurase
IPR005303 MOSC_N
IPR015424 PyrdxlP-dep_Trfase
IPR015422 PyrdxlP-dep_Trfase_dom1
IPR015421 PyrdxlP-dep_Trfase_major
IPR011037 Pyrv_Knase-like_insert_dom_sf
PfamiView protein in Pfam
PF00266 Aminotran_5, 1 hit
PF03473 MOSC, 1 hit
PF03476 MOSC_N, 1 hit
SUPFAMiSSF50800 SSF50800, 1 hit
SSF53383 SSF53383, 2 hits
PROSITEiView protein in PROSITE
PS51340 MOSC, 1 hit

Sequencei

Sequence statusi: Complete.

Q96EN8-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAGAAAESGR ELWTFAGSRD PSAPRLAYGY GPGSLRELRA REFSRLAGTV
60 70 80 90 100
YLDHAGATLF SQSQLESFTS DLMENTYGNP HSQNISSKLT HDTVEQVRYR
110 120 130 140 150
ILAHFHTTAE DYTVIFTAGS TAALKLVAEA FPWVSQGPES SGSRFCYLTD
160 170 180 190 200
SHTSVVGMRN VTMAINVIST PVRPEDLWSA EERSASASNP DCQLPHLFCY
210 220 230 240 250
PAQSNFSGVR YPLSWIEEVK SGRLHPVSTP GKWFVLLDAA SYVSTSPLDL
260 270 280 290 300
SAHQADFVPI SFYKIFGFPT GLGALLVHNR AAPLLRKTYF GGGTASAYLA
310 320 330 340 350
GEDFYIPRQS VAQRFEDGTI SFLDVIALKH GFDTLERLTG GMENIKQHTF
360 370 380 390 400
TLAQYTYVAL SSLQYPNGAP VVRIYSDSEF SSPEVQGPII NFNVLDDKGN
410 420 430 440 450
IIGYSQVDKM ASLYNIHLRT GCFCNTGACQ RHLGISNEMV RKHFQAGHVC
460 470 480 490 500
GDNMDLIDGQ PTGSVRISFG YMSTLDDVQA FLRFIIDTRL HSSGDWPVPQ
510 520 530 540 550
AHADTGETGA PSADSQADVI PAVMGRRSLS PQEDALTGSR VWNNSSTVNA
560 570 580 590 600
VPVAPPVCDV ARTQPTPSEK AAGVLEGALG PHVVTNLYLY PIKSCAAFEV
610 620 630 640 650
TRWPVGNQGL LYDRSWMVVN HNGVCLSQKQ EPRLCLIQPF IDLRQRIMVI
660 670 680 690 700
KAKGMEPIEV PLEENSERTQ IRQSRVCADR VSTYDCGEKI SSWLSTFFGR
710 720 730 740 750
PCHLIKQSSN SQRNAKKKHG KDQLPGTMAT LSLVNEAQYL LINTSSILEL
760 770 780 790 800
HRQLNTSDEN GKEELFSLKD LSLRFRANII INGKRAFEEE KWDEISIGSL
810 820 830 840 850
RFQVLGPCHR CQMICIDQQT GQRNQHVFQK LSESRETKVN FGMYLMHASL
860 870 880
DLSSPCFLSV GSQVLPVLKE NVEGHDLPAS EKHQDVTS
Length:888
Mass (Da):98,120
Last modified:May 18, 2010 - v2
Checksum:i07DB9457516E8C06
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti119G → E in BAD96606 (PubMed:14702039).Curated1
Sequence conflicti689K → E in BAD96606 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_02752857A → P in XAN2. 1 PublicationCorresponds to variant dbSNP:rs886037854EnsemblClinVar.1
Natural variantiVAR_027529120S → N. Corresponds to variant dbSNP:rs3744900Ensembl.1
Natural variantiVAR_027530170T → I2 PublicationsCorresponds to variant dbSNP:rs623053Ensembl.1
Natural variantiVAR_027531184S → G3 PublicationsCorresponds to variant dbSNP:rs540967Ensembl.1
Natural variantiVAR_027532225H → R4 PublicationsCorresponds to variant dbSNP:rs623558Ensembl.1
Natural variantiVAR_027533294T → I in XAN2. 1 PublicationCorresponds to variant dbSNP:rs577279030Ensembl.1
Natural variantiVAR_027534358V → M3 PublicationsCorresponds to variant dbSNP:rs678560Ensembl.1
Natural variantiVAR_027535495D → N. Corresponds to variant dbSNP:rs8088347Ensembl.1
Natural variantiVAR_027536541V → L. Corresponds to variant dbSNP:rs672924Ensembl.1
Natural variantiVAR_027537703H → N2 PublicationsCorresponds to variant dbSNP:rs594445Ensembl.1
Natural variantiVAR_045899776R → C in XAN2. 1 PublicationCorresponds to variant dbSNP:rs750896617EnsemblClinVar.1
Natural variantiVAR_027538867V → A. Corresponds to variant dbSNP:rs1057251Ensembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK000740 mRNA Translation: BAA91353.1
AK222886 mRNA Translation: BAD96606.1
AC023043 Genomic DNA No translation available.
BC012079 mRNA Translation: AAH12079.1
AL834481 mRNA Translation: CAD39140.1
CCDSiCCDS11919.1
PIRiJC7680
RefSeqiNP_060417.2, NM_017947.2
UniGeneiHs.405028

Genome annotation databases

EnsembliENST00000261326; ENSP00000261326; ENSG00000075643
GeneIDi55034
KEGGihsa:55034
UCSCiuc002kzq.5 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Entry informationi

Entry nameiMOCOS_HUMAN
AccessioniPrimary (citable) accession number: Q96EN8
Secondary accession number(s): Q53GP5, Q8N3A4, Q9NWM7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: September 19, 2006
Last sequence update: May 18, 2010
Last modified: July 18, 2018
This is version 139 of the entry and version 2 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

Complete proteome, Reference proteome

Documents

  1. Human chromosome 18
    Human chromosome 18: 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. SIMILARITY comments
    Index of protein domains and families

We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

Do not show this banner again
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health