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Record Information
Creation Date2006-05-22 14:17:41 UTC
Update Date2017-03-02 21:26:56 UTC
Secondary Accession NumbersNone
Metabolite Identification
Common NameMolybdopterin
DescriptionMolybdenum cofactor is a cofactor required for the activity of enzymes such as sulfite oxidase, xanthine oxidoreductase, and aldehyde oxidase. It is a coordination complex formed between molybdopterin (which, despite the name, does not contain molybdenum) and an oxide of molybdenum. Molybdopterins, in turn, are synthesized from guanosine triphosphate. Molybdenum cofactor functions directly in ethylbenzene dehydrogenase, glyceraldehyde-3-phosphate ferredoxin oxidoreductase, and respiratory arsenate reductase. In animals and plants these enzymes use molybdenum bound at the active site in a tricyclic molybdenum cofactor. All molybdenum-using enzymes so far identified in nature use this cofactor The simplest structure of molybdopterin contains a pyranopterin coordinated to molybdenum. The pyranopterin structure is a fused ring system containing a pyran fused to pterin. In addition, the pyran ring is substituted with two thiols and an alkyl phosphate. In molybdopterin, the thiols coordinate to molybdenum. In some cases, the alkyl phosphate group is replaced by an alkyl diphosphate nucleotide. -- Wikipedia.
Molybdenum cofactorHMDB
Molybdenum enzyme molybdenum cofactorHMDB
Molybdoenzyme molybdenum-containing cofactorHMDB
Nitrate reductase molybdenum cofactorHMDB
Pterin molybdenum cofactorHMDB
moco CofactorMeSH
Molybdopterin cofactorMeSH
Chemical FormulaC10H12MoN5O8PS2
Average Molecular Weight521.27
Monoisotopic Molecular Weight522.891898123
IUPAC Name({5-amino-7,13,13-trioxo-17-oxa-12,14-dithia-2,4,6,9-tetraaza-13-molybdatetracyclo[³,⁸.0¹¹,¹⁵]heptadeca-3(8),4,11(15)-trien-16-yl}methoxy)phosphonic acid
Traditional Name{5-amino-7,13,13-trioxo-17-oxa-12,14-dithia-2,4,6,9-tetraaza-13-molybdatetracyclo[³,⁸.0¹¹,¹⁵]heptadeca-3(8),4,11(15)-trien-16-yl}methoxyphosphonic acid
CAS Registry Number73508-07-3
InChI Identifier
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as molybdopterins. These are cofactors or analogs thereof, with a structure based on a furan ring fused to a pterin. In addition, the pyran ring features two thiolates, which serve as ligands in molybdo- and tungstoenzymes. In some cases, the alkyl phosphate group is replaced by an alkyl diphosphate nucleotide.
KingdomChemical entities
Super ClassOrganic compounds
ClassOrganoheterocyclic compounds
Sub ClassPteridines and derivatives
Direct ParentMolybdopterins
Alternative Parents
  • Molybdopterin
  • Pyranopterin
  • Aminopyrimidine
  • Pyrimidone
  • Secondary aliphatic/aromatic amine
  • Monoalkyl phosphate
  • Organic phosphoric acid derivative
  • Phosphoric acid ester
  • Primary aromatic amine
  • Pyran
  • Pyrimidine
  • Alkyl phosphate
  • Heteroaromatic compound
  • Vinylogous amide
  • Oxacycle
  • Azacycle
  • Organic transition metal salt
  • Secondary amine
  • Organic nitrogen compound
  • Amine
  • Hydrocarbon derivative
  • Primary amine
  • Organic oxide
  • Organosulfur compound
  • Organooxygen compound
  • Organonitrogen compound
  • Organopnictogen compound
  • Organic salt
  • Organic zwitterion
  • Organic oxygen compound
  • Aromatic heteropolycyclic compound
Molecular FrameworkAromatic heteropolycyclic compounds
External DescriptorsNot Available
StatusExpected but not Quantified
  • Endogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locationsNot Available
Physical Properties
Experimental Properties
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility14.9 mg/mLALOGPS
pKa (Strongest Acidic)1.2ChemAxon
pKa (Strongest Basic)3.88ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count11ChemAxon
Hydrogen Donor Count6ChemAxon
Polar Surface Area201.67 Å2ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity100.05 m3·mol-1ChemAxon
Polarizability38.78 Å3ChemAxon
Number of Rings4ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
SpectraNot Available
Biological Properties
Cellular LocationsNot Available
Biofluid LocationsNot Available
Tissue Location
  • Fibroblasts
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase DeficiencySMP00137Not Available
3-Hydroxy-3-Methylglutaryl-CoA Lyase DeficiencySMP00138Not Available
3-hydroxyisobutyric acid dehydrogenase deficiencySMP00521Not Available
3-hydroxyisobutyric aciduriaSMP00522Not Available
3-Methylcrotonyl Coa Carboxylase Deficiency Type ISMP00237Not Available
3-Methylglutaconic Aciduria Type ISMP00139Not Available
3-Methylglutaconic Aciduria Type IIISMP00140Not Available
3-Methylglutaconic Aciduria Type IVSMP00141Not Available
Azathioprine PathwaySMP00427Not Available
Beta-Ketothiolase DeficiencySMP00173Not Available
Caffeine MetabolismSMP00028map00232
Citalopram Metabolism PathwaySMP00627Not Available
Citalopram PathwaySMP00424Not Available
Doxorubicin Metabolism PathwaySMP00650Not Available
HypophosphatasiaSMP00503Not Available
Isobutyryl-coa dehydrogenase deficiencySMP00523Not Available
Isovaleric acidemiaSMP00524Not Available
Isovaleric AciduriaSMP00238Not Available
Maple Syrup Urine DiseaseSMP00199Not Available
Mercaptopurine Metabolism PathwaySMP00609Not Available
Mercaptopurine PathwaySMP00428Not Available
Methylmalonate Semialdehyde Dehydrogenase DeficiencySMP00384Not Available
Methylmalonic AciduriaSMP00200Not Available
Nicotinate and Nicotinamide MetabolismSMP00048map00760
Nicotine Metabolism PathwaySMP00628Not Available
Nicotine PathwaySMP00431Not Available
Propionic AcidemiaSMP00236Not Available
Pterine BiosynthesisSMP00005map00790
Thioguanine PathwaySMP00430Not Available
Tryptophan MetabolismSMP00063map00380
Valine, Leucine and Isoleucine DegradationSMP00032map00280
Vitamin B6 MetabolismSMP00017map00750
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022906
KNApSAcK IDNot Available
Chemspider ID4883416
KEGG Compound IDC05924
BioCyc IDCPD0-1882
BiGG IDNot Available
Wikipedia LinkMolybdopterin
NuGOwiki LinkHMDB02206
Metagene LinkHMDB02206
PubChem Compound23304237
PDB IDNot Available
ChEBI ID21437
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Reiss J, Gross-Hardt S, Christensen E, Schmidt P, Mendel RR, Schwarz G: A mutation in the gene for the neurotransmitter receptor-clustering protein gephyrin causes a novel form of molybdenum cofactor deficiency. Am J Hum Genet. 2001 Jan;68(1):208-13. Epub 2000 Nov 28. [PubMed:11095995 ]
  2. Schwarz G: Molybdenum cofactor biosynthesis and deficiency. Cell Mol Life Sci. 2005 Dec;62(23):2792-810. [PubMed:16261263 ]
  3. Macaya A, Brunso L, Fernandez-Castillo N, Arranz JA, Ginjaar HB, Cuenca-Leon E, Corominas R, Roig M, Cormand B: Molybdenum cofactor deficiency presenting as neonatal hyperekplexia: a clinical, biochemical and genetic study. Neuropediatrics. 2005 Dec;36(6):389-94. [PubMed:16429380 ]
  4. Bamforth FJ, Johnson JL, Davidson AG, Wong LT, Lockitch G, Applegarth DA: Biochemical investigation of a child with molybdenum cofactor deficiency. Clin Biochem. 1990 Dec;23(6):537-42. [PubMed:2289312 ]
  5. Mize C, Johnson JL, Rajagopalan KV: Defective molybdopterin biosynthesis: clinical heterogeneity associated with molybdenum cofactor deficiency. J Inherit Metab Dis. 1995;18(3):283-90. [PubMed:7474893 ]


General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
Uniprot ID:
Molecular weight:
General function:
Involved in oxidoreductase activity
Specific function:
Key enzyme in purine degradation. Catalyzes the oxidation of hypoxanthine to xanthine. Catalyzes the oxidation of xanthine to uric acid. Contributes to the generation of reactive oxygen species. Has also low oxidase activity towards aldehydes (in vitro).
Gene Name:
Uniprot ID:
Molecular weight:
General function:
Involved in Mo-molybdopterin cofactor biosynthetic process
Specific 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.
Gene Name:
Uniprot ID:
Molecular weight:
Cyclic pyranopterin monophosphate + Sulfur donor → Molybdopterindetails
General function:
Involved in Mo-molybdopterin cofactor biosynthetic process
Specific 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.
Gene Name:
Uniprot ID:
Molecular weight:
Cyclic pyranopterin monophosphate + [molybdopterin-synthase sulfur-carrier protein]-Gly-NH-CH(2)-C(O)SH + Water → Molybdopterin + [molybdopterin-synthase sulfur-carrier protein]details
Cyclic pyranopterin monophosphate + Sulfur donor → Molybdopterindetails
General function:
Involved in catalytic activity
Specific function:
Plays a central role in 2-thiolation of mcm(5)S(2)U at tRNA wobble positions of tRNA(Lys), tRNA(Glu) and tRNA(Gln). Also essential during biosynthesis of the molybdenum cofactor. Acts by mediating the C-terminal thiocarboxylation of sulfur carriers URM1 and MOCS2A. Its N-terminus first activates URM1 and MOCS2A as acyl-adenylates (-COAMP), then the persulfide sulfur on the catalytic cysteine is transferred to URM1 and MOCS2A to form thiocarboxylation (-COSH) of their C-terminus. The reaction probably involves hydrogen sulfide that is generated from the persulfide intermediate and that acts as nucleophile towards URM1 and MOCS2A. Subsequently, a transient disulfide bond is formed. Does not use thiosulfate as sulfur donor; NFS1 probably acting as a sulfur donor for thiocarboxylation reactions.
Gene Name:
Uniprot ID:
Molecular weight:
General function:
Involved in Mo-molybdopterin cofactor biosynthetic process
Specific function:
Microtubule-associated protein involved in membrane protein-cytoskeleton interactions. It is thought to anchor the inhibitory glycine receptor (GLYR) to subsynaptic microtubules (By similarity). Catalyzes two steps in the biosynthesis of the molybdenum cofactor. In the first step, molybdopterin is adenylated. Subsequently, molybdate is inserted into adenylated molybdopterin and AMP is released.
Gene Name:
Uniprot ID:
Molecular weight:
Adenosine triphosphate + Molybdopterin → Pyrophosphate + Adenylyl-molybdopterindetails
Adenylyl-molybdopterin + Molybdate → Molybdopterin + Adenosine monophosphatedetails
General function:
Involved in metabolic process
Specific 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.
Gene Name:
Uniprot ID:
Molecular weight:
Molybdopterin + L-Cysteine + Hydrogen Ion → thio-molybdenum cofactor + L-Alanine + Waterdetails