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Aliases for MOCS2 Gene

Aliases for MOCS2 Gene

  • Molybdenum Cofactor Synthesis 2 2 3
  • MOCODB 3 6
  • MOCS2A 3 4
  • MOCS2B 3 4
  • MCBPE 3 4
  • MOCO1 3 4
  • MPTS 3 6
  • Molybdenum Cofactor Synthesis Protein 2 Large Subunit 4
  • Molybdenum Cofactor Synthesis Protein 2 Small Subunit 4
  • Molybdopterin Synthase Small And Large Subunit 3
  • Molybdopterin Synthase Sulfur Carrier Subunit 3
  • Molybdenum Cofactor Biosynthesis Protein E 3
  • Molybdenum Cofactor Synthesis Protein 2A 4
  • Molybdenum Cofactor Synthesis Protein 2B 4
  • Molybdopterin-Synthase Large Subunit 4
  • Molybdopterin-Synthase Small Subunit 4
  • Sulfur Carrier Protein MOCS2A 4
  • MPT Synthase Large Subunit 4
  • EC 2.8.1.12 4
  • MOCO1-A 4
  • MOCO1-B 4

External Ids for MOCS2 Gene

Previous GeneCards Identifiers for MOCS2 Gene

  • GC05M052558
  • GC05M053821
  • GC05M052364
  • GC05M052409
  • GC05M052429
  • GC05M049365

Summaries for MOCS2 Gene

Entrez Gene Summary for MOCS2 Gene

  • Eukaryotic molybdoenzymes use a unique molybdenum cofactor (MoCo) consisting of a pterin, termed molybdopterin, and the catalytically active metal molybdenum. MoCo is synthesized from precursor Z by the heterodimeric enzyme molybdopterin synthase. The large and small subunits of molybdopterin synthase are both encoded from this gene by overlapping open reading frames. The proteins were initially thought to be encoded from a bicistronic transcript. They are now thought to be encoded from monocistronic transcripts. Alternatively spliced transcripts have been found for this locus that encode the large and small subunits. [provided by RefSeq, Jul 2008]

GeneCards Summary for MOCS2 Gene

MOCS2 (Molybdenum Cofactor Synthesis 2) is a Protein Coding gene. Diseases associated with MOCS2 include molybdenum cofactor deficiency b and molybdenum cofactor deficiency. Among its related pathways are Disease and Metabolism. GO annotations related to this gene include identical protein binding and transferase activity.

UniProtKB/Swiss-Prot for MOCS2 Gene

  • Acts as a sulfur carrier required for molybdopterin biosynthesis. Component of the molybdopterin synthase complex that catalyzes the conversion of precursor Z into molybdopterin by mediating the incorporation of 2 sulfur atoms into precursor Z to generate a dithiolene group. In the complex, serves as sulfur donor by being thiocarboxylated (-COSH) at its C-terminus by MOCS3. After interaction with MOCS2B, the sulfur is then transferred to precursor Z to form molybdopterin.

  • 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.

Gene Wiki entry for MOCS2 Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for MOCS2 Gene

Genomics for MOCS2 Gene

Regulatory Elements for MOCS2 Gene

Epigenetics Products

  • DNA Methylation CpG Assay Predesigned for Pyrosequencing in human,mouse,rat

Genomic Location for MOCS2 Gene

Start:
53,095,679 bp from pter
End:
53,110,063 bp from pter
Size:
14,385 bases
Orientation:
Minus strand

Genomic View for MOCS2 Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for MOCS2 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for MOCS2 Gene

Proteins for MOCS2 Gene

  • Protein details for MOCS2 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    O96007-MOC2B_HUMAN
    Recommended name:
    Molybdopterin synthase catalytic subunit
    Protein Accession:
    O96007
    Secondary Accessions:
    • Q6IAI3

    Protein attributes for MOCS2 Gene

    Size:
    188 amino acids
    Molecular mass:
    20944 Da
    Quaternary structure:
    • Heterotetramer; composed of 2 small (MOCS2A) and 2 large (MOCS2B) subunits.
    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)

    Three dimensional structures from OCA and Proteopedia for MOCS2 Gene

  • Protein details for MOCS2 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    O96033-MOC2A_HUMAN
    Recommended name:
    Molybdopterin synthase sulfur carrier subunit
    Protein Accession:
    O96033
    Secondary Accessions:

    Protein attributes for MOCS2 Gene

    Size:
    88 amino acids
    Molecular mass:
    9755 Da
    Quaternary structure:
    • Heterotetramer; composed of 2 small (MOCS2A) and 2 large (MOCS2B) subunits.
    Miscellaneous:
    • This protein is produced by a bicistronic gene which also produces the large subunit (MOCS2B) 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)

neXtProt entry for MOCS2 Gene

Proteomics data for MOCS2 Gene at MOPED

Post-translational modifications for MOCS2 Gene

  • C-terminal thiocarboxylation occurs in 2 steps, it is first acyl-adenylated (-COAMP) via the hesA/moeB/thiF part of MOCS3, then thiocarboxylated (-COSH) via the rhodanese domain of MOCS3.
  • Modification sites at PhosphoSitePlus
  • Modification sites at neXtProt

Other Protein References for MOCS2 Gene

No data available for DME Specific Peptides for MOCS2 Gene

Domains for MOCS2 Gene

Suggested Antigen Peptide Sequences for MOCS2 Gene

Graphical View of Domain Structure for InterPro Entry

O96033

UniProtKB/Swiss-Prot:

MOC2A_HUMAN :
  • O96033
Family:
  • Belongs to the MoaD family. MOCS2A subfamily.
  • Belongs to the MoaE family. MOCS2B subfamily.
genes like me logo Genes that share domains with MOCS2: view

No data available for Gene Families for MOCS2 Gene

Function for MOCS2 Gene

Molecular function for MOCS2 Gene

GENATLAS Biochemistry: molybdopterine synthase,converting precursor Z (see MOCS1) to molybdopterine,predominantly expressed in heart and skeletal muscle,catalyzing the second step of the molybdenum cofactor synthesis,including the small MOCS2A and the large MOCS2B subunits,transcript by a single bicistronic mRNA with overlapping reading frames
UniProtKB/Swiss-Prot Function: Acts as a sulfur carrier required for molybdopterin biosynthesis. Component of the molybdopterin synthase complex that catalyzes the conversion of precursor Z into molybdopterin by mediating the incorporation of 2 sulfur atoms into precursor Z to generate a dithiolene group. In the complex, serves as sulfur donor by being thiocarboxylated (-COSH) at its C-terminus by MOCS3. After interaction with MOCS2B, the sulfur is then transferred to precursor Z to form molybdopterin.
UniProtKB/Swiss-Prot CatalyticActivity: Cyclic pyranopterin phosphate + 2 [molybdopterin-synthase sulfur-carrier protein]-Gly-NH-CH(2)-C(O)SH + H(2)O = molybdopterin + 2 [molybdopterin-synthase sulfur-carrier protein].
UniProtKB/Swiss-Prot 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.

Enzyme Numbers (IUBMB) for MOCS2 Gene

Gene Ontology (GO) - Molecular Function for MOCS2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0000166 nucleotide binding IEA --
GO:0030366 molybdopterin synthase activity TAS --
GO:0042802 identical protein binding IEA --
genes like me logo Genes that share ontologies with MOCS2: view

Animal Model Products

CRISPR Products

miRNA Products

Inhibitory RNA Products

  • Predesigned siRNA for gene silencing in human,mouse,rat for MOCS2

In Situ Assay Products

Flow Cytometry Products

No data available for Phenotypes , Animal Models , miRNA , Transcription Factor Targeting and HOMER Transcription for MOCS2 Gene

Localization for MOCS2 Gene

Subcellular locations from UniProtKB/Swiss-Prot for MOCS2 Gene

Subcellular locations from

COMPARTMENTS
Jensen Localization Image for MOCS2 Gene COMPARTMENTS Subcellular localization image for MOCS2 gene
Compartment Confidence
cytosol 5
nucleus 4
peroxisome 2
endoplasmic reticulum 1
mitochondrion 1
plasma membrane 1

Gene Ontology (GO) - Cellular Components for MOCS2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005634 nucleus IDA --
GO:0005737 cytoplasm IDA --
GO:0005829 cytosol TAS --
GO:0019008 molybdopterin synthase complex NAS 9889283
GO:0070062 extracellular exosome IDA 23376485
genes like me logo Genes that share ontologies with MOCS2: view

Pathways for MOCS2 Gene

SuperPathways for MOCS2 Gene

Superpath Contained pathways
1 Defective BTD causes biotidinase deficiency
2 Metabolism
3 Disease
4 Folate biosynthesis
5 Sulfur relay system
genes like me logo Genes that share pathways with MOCS2: view

UniProtKB/Swiss-Prot O96033-MOC2A_HUMAN

  • Pathway: Cofactor biosynthesis; molybdopterin biosynthesis.

Interacting Proteins for MOCS2 Gene

Gene Ontology (GO) - Biological Process for MOCS2 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 IMP 10053004
GO:0032324 molybdopterin cofactor biosynthetic process TAS --
GO:0044281 small molecule metabolic process TAS --
genes like me logo Genes that share ontologies with MOCS2: view

Compounds for MOCS2 Gene

(1) HMDB Compounds for MOCS2 Gene

Compound Synonyms Cas Number PubMed IDs
Molybdopterin
  • MoCo
73508-07-3

(6) Novoseek inferred chemical compound relationships for MOCS2 Gene

Compound -log(P) Hits PubMed IDs
molybdopterin 97.1 43
moco 95.8 7
moae 95.2 3
molybdenum 89.8 9
pterin 75.9 2
genes like me logo Genes that share compounds with MOCS2: view

Transcripts for MOCS2 Gene

Unigene Clusters for MOCS2 Gene

Molybdenum cofactor synthesis 2:
Representative Sequences:

CRISPR Products

miRNA Products

Inhibitory RNA Products

  • Predesigned siRNA for gene silencing in human,mouse,rat for MOCS2

Primer Products

Flow Cytometry Products

Alternative Splicing Database (ASD) splice patterns (SP) for MOCS2 Gene

ExUns: 1a · 1b ^ 2 ^ 3 ^ 4a · 4b ^ 5 ^ 6a · 6b ^ 7 ^ 8a · 8b
SP1: - -
SP2: -
SP3: - - - -
SP4:
SP5:

Relevant External Links for MOCS2 Gene

GeneLoc Exon Structure for
MOCS2
ECgene alternative splicing isoforms for
MOCS2

Expression for MOCS2 Gene

mRNA expression in normal human tissues for MOCS2 Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

Integrated Proteomics: protein expression from ProteomicsDB, PaxDb, MOPED, and MaxQB for MOCS2 Gene

SOURCE GeneReport for Unigene cluster for MOCS2 Gene Hs.163645

mRNA Expression by UniProt/SwissProt for MOCS2 Gene

O96007-MOC2B_HUMAN
Tissue specificity: Widely expressed. 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.
genes like me logo Genes that share expressions with MOCS2: view

In Situ Assay Products

No data available for mRNA differential expression in normal tissues for MOCS2 Gene

Orthologs for MOCS2 Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for MOCS2 Gene

Organism Taxonomy Gene Similarity Type Details
chimpanzee
(Pan troglodytes)
Mammalia MOCS2 35
  • 99.65 (n)
  • 99.47 (a)
MOCS2 36
  • 99 (a)
OneToOne
cow
(Bos Taurus)
Mammalia MOCS2 35
  • 90.2 (n)
  • 87.17 (a)
MOCS2 36
  • 87 (a)
OneToOne
dog
(Canis familiaris)
Mammalia MOCS2 35
  • 89.3 (n)
  • 86.63 (a)
MOCS2 36
  • 81 (a)
OneToOne
mouse
(Mus musculus)
Mammalia Mocs2 35
  • 85.03 (n)
  • 83.42 (a)
Mocs2 16
Mocs2 36
  • 82 (a)
OneToOne
oppossum
(Monodelphis domestica)
Mammalia MOCS2 36
  • 73 (a)
OneToOne
platypus
(Ornithorhynchus anatinus)
Mammalia MOCS2 36
  • 68 (a)
OneToOne
rat
(Rattus norvegicus)
Mammalia Mocs2 35
  • 84.67 (n)
  • 85.03 (a)
chicken
(Gallus gallus)
Aves MOCS2 35
  • 72.15 (n)
  • 73.03 (a)
MOCS2 36
  • 72 (a)
OneToOne
lizard
(Anolis carolinensis)
Reptilia MOCS2 36
  • 69 (a)
OneToOne
tropical clawed frog
(Silurana tropicalis)
Amphibia mocs2 35
  • 69.7 (n)
  • 64.39 (a)
Str.7298 35
zebrafish
(Danio rerio)
Actinopterygii Dr.16660 35
mocs2 35
  • 61.94 (n)
  • 63.51 (a)
mocs2 36
  • 59 (a)
OneToOne
African malaria mosquito
(Anopheles gambiae)
Insecta MOC2B_ANOGA 35
  • 52.96 (n)
  • 54.61 (a)
fruit fly
(Drosophila melanogaster)
Insecta Mocs2 35
  • 55.72 (n)
  • 57.66 (a)
Mocs2 36
  • 22 (a)
OneToOne
CG10238 37
  • 56 (a)
worm
(Caenorhabditis elegans)
Secernentea T27A3.6 35
  • 50.7 (n)
  • 47.18 (a)
T27A3.6 36
  • 20 (a)
OneToOne
thale cress
(Arabidopsis thaliana)
eudicotyledons AT2G43760 35
  • 54.11 (n)
  • 52.17 (a)
rice
(Oryza sativa)
Liliopsida Os02g0140300 35
  • 44.6 (n)
  • 42.25 (a)
bread mold
(Neurospora crassa)
Ascomycetes NCU03170 35
  • 46.42 (n)
  • 43.7 (a)
sea squirt
(Ciona intestinalis)
Ascidiacea Cin.4481 35
sea squirt
(Ciona savignyi)
Ascidiacea CSA.3820 36
  • 52 (a)
OneToOne
Species with no ortholog for MOCS2:
  • 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)
  • 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 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)
  • wheat (Triticum aestivum)

Evolution for MOCS2 Gene

ENSEMBL:
Gene Tree for MOCS2 (if available)
TreeFam:
Gene Tree for MOCS2 (if available)

Paralogs for MOCS2 Gene

No data available for Paralogs for MOCS2 Gene

Variants for MOCS2 Gene

Sequence variations from dbSNP and Humsavar for MOCS2 Gene

SNP ID Clin Chr 05 pos Sequence Context AA Info Type MAF
rs10120 -- 53,098,224(+) GGGAT(A/G)GGGGG utr-variant-3-prime
rs12887 -- 53,098,171(+) GTTCT(G/T)CCAAC utr-variant-3-prime
rs30163 -- 53,105,990(-) ctaat(A/G)atcag intron-variant
rs30164 -- 53,102,901(-) gatca(A/G)tttca intron-variant
rs1057555 -- 53,095,755(-) CAGGT(A/C)TTCCT utr-variant-3-prime

Structural Variations from Database of Genomic Variants (DGV) for MOCS2 Gene

Variant ID Type Subtype PubMed ID
nsv830296 CNV Gain 17160897
nsv442948 CNV CNV 18776908

Relevant External Links for MOCS2 Gene

HapMap Linkage Disequilibrium report
MOCS2
Human Gene Mutation Database (HGMD)
MOCS2

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for MOCS2 Gene

Disorders for MOCS2 Gene

(1) OMIM Diseases for MOCS2 Gene (603708)

UniProtKB/Swiss-Prot

MOC2A_HUMAN
  • 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. {ECO:0000269 PubMed:10053004, ECO:0000269 PubMed:11746050, ECO:0000269 PubMed:16021469, ECO:0000269 PubMed:16737835}. Note=The disease is caused by mutations affecting the gene represented in this entry.
  • 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. {ECO:0000269 PubMed:10053004, ECO:0000269 PubMed:16021469, ECO:0000269 PubMed:16737835}. Note=The disease is caused by mutations affecting the gene represented in this entry.

(1) Novoseek inferred disease relationships for MOCS2 Gene

Disease -log(P) Hits PubMed IDs
molybdenum cofactor deficiency 95.4 2
genes like me logo Genes that share disorders with MOCS2: view

Publications for MOCS2 Gene

  1. Human molybdopterin synthase gene: genomic structure and mutations in molybdenum cofactor deficiency type B. (PMID: 10053004) Reiss J. … Zabot M.-T. (Am. J. Hum. Genet. 1999) 2 3 4 23
  2. The two subunits of human molybdopterin synthase: evidence for a bicistronic messenger RNA with overlapping reading frames. (PMID: 9889283) Sloan J. … Unkles S.E. (Nucleic Acids Res. 1999) 2 3 4 23
  3. Human molybdopterin synthase gene: identification of a bicistronic transcript with overlapping reading frames. (PMID: 10053003) Stallmeyer B. … Mendel R.R. (Am. J. Hum. Genet. 1999) 3 4 23
  4. Mechanistic studies of human molybdopterin synthase reaction and characterization of mutants identified in group B patients of molybdenum cofactor deficiency. (PMID: 12732628) Leimkuehler S. … Mendel R.R. (J. Biol. Chem. 2003) 3 4 23
  5. Evidence for the physiological role of a rhodanese-like protein for the biosynthesis of the molybdenum cofactor in humans. (PMID: 15073332) Matthies A. … Leimkuehler S. (Proc. Natl. Acad. Sci. U.S.A. 2004) 3 4 23

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