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Record Information
Version3.6
Creation Date2006-05-22 14:17:41 UTC
Update Date2017-09-14 00:33:58 UTC
HMDB IDHMDB0002206
Secondary Accession Numbers
  • HMDB02206
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.
Structure
Thumb
SynonymsNot Available
Chemical FormulaC10H16MoN5O8PS2
Average Molecular Weight525.31
Monoisotopic Molecular Weight526.923197
IUPAC Namemolybdenum(2+) ion (5aR,8R,9aR)-8-[(hydrogen phosphonatooxy)methyl]-2-imino-6,7-disulfanyl-1H,2H,5H,5aH,8H,9aH,10H-pyrano[3,2-g]pteridin-4-olate dihydrate
Traditional Namemolybdenum(2+) ion (5aR,8R,9aR)-8-[(hydrogen phosphonatooxy)methyl]-2-imino-6,7-disulfanyl-1H,5H,5aH,8H,9aH,10H-pyrano[3,2-g]pteridin-4-olate dihydrate
CAS Registry Number73508-07-3
SMILES
O.O.[Mo++].[H]C1(COP(O)([O-])=O)OC2([H])NC3=C(NC2([H])C(S)=C1S)C([O-])=NC(=N)N3
InChI Identifier
InChI=1S/C10H14N5O6PS2.Mo.2H2O/c11-10-14-7-4(8(16)15-10)12-3-6(24)5(23)2(21-9(3)13-7)1-20-22(17,18)19;;;/h2-3,9,12,23-24H,1H2,(H2,17,18,19)(H4,11,13,14,15,16);;2*1H2/q;+2;;/p-2
InChI KeyVUKICSJFFDCESC-UHFFFAOYSA-L
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as pyranopterins and derivatives. These are pterin derivatives in which a pyran ring is fused either to the pyrimidine ring or the pyrazine ring of the pterin moiety.
KingdomOrganic compounds
Super ClassOrganoheterocyclic compounds
ClassPteridines and derivatives
Sub ClassPterins and derivatives
Direct ParentPyranopterins and derivatives
Alternative Parents
Substituents
  • Pyranopterin
  • Secondary aliphatic/aromatic amine
  • Organic phosphate
  • Organic phosphoric acid derivative
  • Phosphoric acid ester
  • Pyran
  • Pyrimidine
  • Alkyl phosphate
  • Heteroaromatic compound
  • Alkylthiol
  • Oxacycle
  • Azacycle
  • Organic transition metal salt
  • Secondary amine
  • Thioenol
  • Amine
  • Organosulfur compound
  • Organooxygen compound
  • Organic oxide
  • Organic salt
  • Organic zwitterion
  • Hydrocarbon derivative
  • Organonitrogen compound
  • Aromatic heteropolycyclic compound
Molecular FrameworkAromatic heteropolycyclic compounds
External DescriptorsNot Available
Ontology
StatusExpected but not Quantified
Origin
  • Endogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locationsNot Available
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility14.9 mg/mLALOGPS
logP0.86ALOGPS
logP-1.9ChemAxon
logS-2.7ALOGPS
pKa (Strongest Acidic)1.19ChemAxon
pKa (Strongest Basic)2.91ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count10ChemAxon
Hydrogen Donor Count7ChemAxon
Polar Surface Area174.18 Å2ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity119.21 m3·mol-1ChemAxon
Polarizability33.04 Å3ChemAxon
Number of Rings3ChemAxon
Bioavailability0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-00di-0001490000-bf4dfec34731bf10ebadView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00di-1606490000-e83ef8869348bf49af28View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-020r-4982200000-b29a94b7f07e6ea22651View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-05e9-9606040000-926bcbb9296f4a9cd5e0View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-004i-9101010000-2dd652c6c798de5919fbView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-004i-9000000000-58324c83b689024dc935View in MoNA
Biological Properties
Cellular LocationsNot Available
Biofluid LocationsNot Available
Tissue Location
  • Fibroblasts
Pathways
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase DeficiencyPw000061Pw000061 greyscalePw000061 simpleNot Available
3-Hydroxy-3-Methylglutaryl-CoA Lyase DeficiencyPw000063Pw000063 greyscalePw000063 simpleNot Available
3-Methylcrotonyl Coa Carboxylase Deficiency Type IPw000065Pw000065 greyscalePw000065 simpleNot Available
3-Methylglutaconic Aciduria Type IPw000066Pw000066 greyscalePw000066 simpleNot Available
3-Methylglutaconic Aciduria Type IIIPw000067Pw000067 greyscalePw000067 simpleNot Available
Displaying entries 1 - 5 of 32 in total
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 LinkHMDB0002206
METLIN ID6546
PubChem Compound23304237
PDB IDNot Available
ChEBI ID21437
References
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 ]

Enzymes

General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
AOX1
Uniprot ID:
Q06278
Molecular weight:
147916.735
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:
XDH
Uniprot ID:
P47989
Molecular weight:
146422.99
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:
MOCS2
Uniprot ID:
O96033
Molecular weight:
9755.235
Reactions
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:
MOCS2
Uniprot ID:
O96007
Molecular weight:
20943.735
Reactions
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:
MOCS3
Uniprot ID:
O95396
Molecular weight:
49668.685
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:
GPHN
Uniprot ID:
Q9NQX3
Molecular weight:
79747.635
Reactions
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:
MOCOS
Uniprot ID:
Q96EN8
Molecular weight:
98118.965
Reactions
Molybdopterin + L-Cysteine + Hydrogen Ion → thio-molybdenum cofactor + L-Alanine + Waterdetails