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Aliases for MOCOS Gene

Aliases for MOCOS Gene

  • Molybdenum Cofactor Sulfurase 2 3
  • MCS 3 4 6
  • HMCS 3 4
  • MOS 3 4
  • Molybdenum Cofactor Sulfurtransferase 4
  • MoCo Sulfurase 4
  • EC 4

External Ids for MOCOS Gene

Summaries for MOCOS Gene

Entrez Gene Summary for MOCOS Gene

  • MOCOS sulfurates the molybdenum cofactor of xanthine dehydrogenase (XDH; MIM 607633) and aldehyde oxidase (AOX1; MIM 602841), which is required for their enzymatic activities (Ichida et al., 2001 [PubMed 11302742]).[supplied by OMIM, Feb 2010]

GeneCards Summary for MOCOS Gene

MOCOS (Molybdenum Cofactor Sulfurase) is a Protein Coding gene. Diseases associated with MOCOS include xanthinuria type 2 and xanthinuria. Among its related pathways are MAPK signaling pathway and Regulation of actin cytoskeleton. GO annotations related to this gene include pyridoxal phosphate binding and molybdenum ion binding. An important paralog of this gene is MARC1.

UniProtKB/Swiss-Prot for MOCOS Gene

  • 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.

Gene Wiki entry for MOCOS Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for MOCOS Gene

Genomics for MOCOS Gene

Genomic Location for MOCOS Gene

36,187,517 bp from pter
36,272,157 bp from pter
84,641 bases
Plus strand

Genomic View for MOCOS Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for MOCOS Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for MOCOS Gene

Regulatory Elements for MOCOS Gene

Proteins for MOCOS Gene

  • Protein details for MOCOS Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Molybdenum cofactor sulfurase
    Protein Accession:
    Secondary Accessions:
    • Q53GP5
    • Q8N3A4
    • Q9NWM7

    Protein attributes for MOCOS Gene

    888 amino acids
    Molecular mass:
    98120 Da
    Name=pyridoxal 5-phosphate; Xref=ChEBI:CHEBI:597326;
    Quaternary structure:
    No Data Available

neXtProt entry for MOCOS Gene

Proteomics data for MOCOS Gene at MOPED

Post-translational modifications for MOCOS Gene

  • Modification sites at PhosphoSitePlus
  • Modification sites at neXtProt

Other Protein References for MOCOS Gene

ENSEMBL proteins:
Reactome Protein details:
REFSEQ proteins:

No data available for DME Specific Peptides for MOCOS Gene

Domains for MOCOS Gene


  • Contains 1 MOSC domain.:
    • Q96EN8
  • Belongs to the class-V pyridoxal-phosphate-dependent aminotransferase family. MOCOS subfamily.:
    • Q96EN8
genes like me logo Genes that share domains with MOCOS: view

No data available for Gene Families for MOCOS Gene

Function for MOCOS Gene

Molecular function for MOCOS Gene

UniProtKB/Swiss-Prot BiophysicochemicalProperties: Kinetic parameters: KM=15 uM for benzamidoxime {ECO:0000269 PubMed:16973608}; Vmax=0.24 nmol/min/mg enzyme {ECO:0000269 PubMed:16973608};
UniProtKB/Swiss-Prot CatalyticActivity: Molybdenum cofactor + L-cysteine + 2 H(+) = thio-molybdenum cofactor + L-alanine + H(2)O.
UniProtKB/Swiss-Prot 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.

Enzyme Numbers (IUBMB) for MOCOS Gene

Gene Ontology (GO) - Molecular Function for MOCOS Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0003824 catalytic activity --
GO:0005515 protein binding IPI 21516116
GO:0008265 Mo-molybdopterin cofactor sulfurase activity TAS --
GO:0016740 transferase activity IEA --
GO:0016829 lyase activity IEA --
genes like me logo Genes that share ontologies with MOCOS: view

miRNA for MOCOS Gene

miRTarBase miRNAs that target MOCOS

No data available for Phenotypes , Animal Models , Transcription Factor Targeting and HOMER Transcription for MOCOS Gene

Localization for MOCOS Gene

Subcellular locations from

Jensen Localization Image for MOCOS Gene COMPARTMENTS Subcellular localization image for MOCOS gene
Compartment Confidence
cytosol 4
nucleus 3
peroxisome 1

Gene Ontology (GO) - Cellular Components for MOCOS Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005575 cellular_component ND --
GO:0005829 cytosol TAS --
genes like me logo Genes that share ontologies with MOCOS: view

Pathways for MOCOS Gene

SuperPathways for MOCOS Gene

Superpath Contained pathways
1 Defective BTD causes biotidinase deficiency
2 MAPK signaling pathway
3 Regulation of actin cytoskeleton
4 Oocyte meiosis
5 Disease
genes like me logo Genes that share pathways with MOCOS: view

Gene Ontology (GO) - Biological Process for MOCOS Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006766 vitamin metabolic process TAS --
GO:0006767 water-soluble vitamin metabolic process TAS --
GO:0006777 Mo-molybdopterin cofactor biosynthetic process IEA --
GO:0008152 metabolic process --
GO:0032324 molybdopterin cofactor biosynthetic process TAS --
genes like me logo Genes that share ontologies with MOCOS: view

Compounds for MOCOS Gene

(3) HMDB Compounds for MOCOS Gene

Compound Synonyms Cas Number PubMed IDs
  • Mo
  • MoCo
Pyridoxal 5'-phosphate
  • Apolon B6

(1) Drugbank Compounds for MOCOS Gene

Compound Synonyms Cas Number Type Actions PubMed IDs
Pyridoxal Phosphate
  • Codecarboxylase
54-47-7 target cofactor

(4) Novoseek inferred chemical compound relationships for MOCOS Gene

Compound -log(P) Hits PubMed IDs
moco 89.2 2
molybdenum 81.8 10
sulfur 56.1 2
cysteine 31.5 2
genes like me logo Genes that share compounds with MOCOS: view

Transcripts for MOCOS Gene

mRNA/cDNA for MOCOS Gene

(1) REFSEQ mRNAs :
(4) Additional mRNA sequences :
(37) Selected AceView cDNA sequences:
(2) Ensembl transcripts including schematic representations, and UCSC links where relevant :

Unigene Clusters for MOCOS Gene

Molybdenum cofactor sulfurase:
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for MOCOS Gene

No ASD Table

Relevant External Links for MOCOS Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for MOCOS Gene

mRNA expression in normal human tissues for MOCOS Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

mRNA differential expression in normal tissues according to GTEx for MOCOS Gene

This gene is overexpressed in Liver (10.0) and Adrenal Gland (6.8).

Integrated Proteomics: protein expression from ProteomicsDB, PaxDb, MOPED, and MaxQB for MOCOS Gene

SOURCE GeneReport for Unigene cluster for MOCOS Gene Hs.405028

genes like me logo Genes that share expressions with MOCOS: view

Orthologs for MOCOS Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for MOCOS Gene

Organism Taxonomy Gene Similarity Type Details
(Pan troglodytes)
Mammalia MOCOS 36
  • 99.4 (n)
  • 99.1 (a)
  • 99 (a)
(Bos Taurus)
Mammalia MOCOS 36
  • 82.99 (n)
  • 80.12 (a)
  • 79 (a)
(Canis familiaris)
Mammalia MOCOS 36
  • 83.86 (n)
  • 80.75 (a)
  • 80 (a)
(Mus musculus)
Mammalia Mocos 36
  • 80.2 (n)
  • 76.78 (a)
Mocos 16
Mocos 37
  • 76 (a)
(Monodelphis domestica)
Mammalia MOCOS 37
  • 65 (a)
(Ornithorhynchus anatinus)
Mammalia MOCOS 37
  • 62 (a)
(Rattus norvegicus)
Mammalia Mocos 36
  • 80.04 (n)
  • 77.2 (a)
(Gallus gallus)
Aves MOCOS 36
  • 65.71 (n)
  • 63.92 (a)
  • 61 (a)
(Anolis carolinensis)
Reptilia MOCOS 37
  • 54 (a)
(Danio rerio)
Actinopterygii mocos 36
  • 55.47 (n)
  • 53.11 (a)
African malaria mosquito
(Anopheles gambiae)
Insecta MOCOS_ANOGA 36
  • 50.71 (n)
  • 44.87 (a)
fruit fly
(Drosophila melanogaster)
Insecta mal 36
  • 50.5 (n)
  • 45.11 (a)
mal 37
  • 38 (a)
(Caenorhabditis elegans)
Secernentea mocs-1 36
  • 47.08 (n)
  • 45.32 (a)
mocs-1 37
  • 35 (a)
thale cress
(Arabidopsis thaliana)
eudicotyledons ABA3 36
  • 47.74 (n)
  • 40.49 (a)
sea squirt
(Ciona savignyi)
Ascidiacea -- 37
  • 41 (a)
Species with no ortholog for MOCOS:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • African clawed frog (Xenopus laevis)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • baker's yeast (Saccharomyces cerevisiae)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • bread mold (Neurospora crassa)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • fission yeast (Schizosaccharomyces pombe)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • K. lactis yeast (Kluyveromyces lactis)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rainbow trout (Oncorhynchus mykiss)
  • rice (Oryza sativa)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • tropical clawed frog (Silurana tropicalis)
  • wheat (Triticum aestivum)

Evolution for MOCOS Gene

Gene Tree for MOCOS (if available)
Gene Tree for MOCOS (if available)

Paralogs for MOCOS Gene

Paralogs for MOCOS Gene

genes like me logo Genes that share paralogs with MOCOS: view

Variants for MOCOS Gene

Sequence variations from dbSNP and Humsavar for MOCOS Gene

SNP ID Clin Chr 18 pos Sequence Context AA Info Type MAF
rs479050 -- 36,241,851(-) tcaga(A/G)ggtgc intron-variant
rs484442 -- 36,249,688(-) tcacc(A/T)gtgtt intron-variant
rs489043 -- 36,250,147(-) ATGGG(C/T)GGGGG intron-variant
rs491339 -- 36,253,278(+) AGAGA(C/T)GTAAT intron-variant
rs503474 -- 36,218,716(+) ggtct(G/T)gctat intron-variant

Structural Variations from Database of Genomic Variants (DGV) for MOCOS Gene

Variant ID Type Subtype PubMed ID
esv987550 CNV Deletion 20482838
nsv522803 CNV Loss 19592680
esv268498 CNV Insertion 20981092
nsv130568 CNV Loss 16902084
esv2667651 CNV Deletion 23128226
nsv909539 CNV Gain 21882294

Relevant External Links for MOCOS Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)

Disorders for MOCOS Gene


  • Xanthinuria 2 (XU2) [MIM:603592]: A 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, patients suffering of xanthinuria 2 cannot metabolize allopurinol into oxypurinol due to dual deficiency of xanthine dehydrogenase and aldehyde oxidase. {ECO:0000269 PubMed:11302742, ECO:0000269 PubMed:14624414, ECO:0000269 PubMed:17368066, ECO:0000269 Ref.11}. Note=The disease is caused by mutations affecting the gene represented in this entry.

(1) Novoseek inferred disease relationships for MOCOS Gene

Disease -log(P) Hits PubMed IDs
xanthinuria, type ii 99 4

Relevant External Links for MOCOS

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
genes like me logo Genes that share disorders with MOCOS: view

Publications for MOCOS Gene

  1. Mutation of human molybdenum cofactor sulfurase gene is responsible to classical xanthinuria type II. (PMID: 11302742) Ichida K. … Nishino T. (Biochem. Biophys. Res. Commun. 2001) 2 3 4 23
  2. 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. (PMID: 17368066) Peretz H. … Landau D. (Mol. Genet. Metab. 2007) 3 4 23
  3. Novel pharmacogenetic markers for treatment outcome in azathioprine-treated inflammatory bowel disease. (PMID: 19500084) Smith M.A. … Sanderson J.D. (Aliment. Pharmacol. Ther. 2009) 3 23 49
  4. The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). (PMID: 15489334) Gerhard D.S. … Malek J. (Genome Res. 2004) 3 4
  5. A probability-based approach for high-throughput protein phosphorylation analysis and site localization. (PMID: 16964243) Beausoleil S.A. … Gygi S.P. (Nat. Biotechnol. 2006) 3 4

Products for MOCOS Gene

Sources for MOCOS Gene

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