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Record Information
Version4.0
StatusExpected but not Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-05-07 19:45:19 UTC
HMDB IDHMDB0001086
Secondary Accession Numbers
  • HMDB01086
Metabolite Identification
Common NameUroporphyrinogen III
DescriptionUroporphyrinogen III, also known as uro'gen III, belongs to the class of organic compounds known as porphyrins. Porphyrins are compounds containing a fundamental skeleton of four pyrrole nuclei united through the alpha-positions by four methine groups to form a macrocyclic structure. Uroporphyrinogen III is a weakly acidic compound (based on its pKa). Uroporphyrinogen III exists in all living species, ranging from bacteria to humans. In humans, uroporphyrinogen III is involved in the metabolic disorder called the acute intermittent porphyria pathway. Outside of the human body, Uroporphyrinogen III has been detected, but not quantified in, several different foods, such as rices, gram beans, garden onions, grapes, and natal plums. This could make uroporphyrinogen III a potential biomarker for the consumption of these foods. The molecular structure of uroporphyrinogen III can be described as a hexahydroporphine core, where each pyrrole ring has the hydrogen atoms on its two outermost carbons replaced by an acetic acid group (−CH2−COOH, "A") and a propionic acid group (−CH2−CH2−COOH, "P"). If uroporphyrinogen-III synthase is not present or inactive, the hydroxymethylbilane will spontaneously cyclise into the structural isomer uroporphyrinogen I, which differs from the III isomer in that the acetic acid ("A") and propionic acid ("P") groups are arranged in a rotationally symmetric order, AP-AP-AP-AP. In the biosynthesis of sirohemes, uroporphyrinogen III is converted by two methyl transferases to dihydrosirohydrochlorin, which is subsequently oxidized sirohydrochlorin, a precursor to the siroheme prosthetic group. It is a colorless compound, like other porphyrinogens. The conversion entails a reversal of the last pyrrole unit (thus swapping the acetic and propionic acid groups) and a condensation reaction that closes the macrocycle by eliminating the final hydroxyl −OH with a hydrogen atom of the first ring. Uroporphyrinogen III is a tetrapyrrole, the first macrocyclic intermediate in the biosynthesis of heme, chlorophyll, vitamin B12, and siroheme. The groups are attached in an asymmetric way: going around the macrocycle, the order is AP-AP-AP-PA.In the general porphyrin biosynthesis pathway, uroporphyrinogen III is derived from the linear tetrapyrrole preuroporphyrinogen (a substituted hydroxymethylbilane) by the action of the enzyme uroporphyrinogen-III cosynthase. In the biosynthesis of hemes and chlorophylls, uroporphyrinogen III is converted into coproporphyrinogen III by the enzyme uroporphyrinogen III decarboxylase.
Structure
Data?1588880719
Synonyms
ValueSource
3,8,13,17-Tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydro-21H,23H-porphine-2,7,12,18-tetrapropanoic acidChEBI
3,8,13,17-Tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,18-tetrapropionic acidChEBI
Uro'gen IIIChEBI
3,8,13,17-Tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydro-21H,23H-porphine-2,7,12,18-tetrapropanoateGenerator
3,8,13,17-Tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,18-tetrapropionateGenerator
3,8,13,17-Tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydro-2,7,12,18-porphinetetrapropionateHMDB
3,8,13,17-Tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydro-2,7,12,18-porphinetetrapropionic acidHMDB
3,8,13,17-Tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,18-tetrapropanoateHMDB
3,8,13,17-Tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,18-tetrapropanoic acidHMDB
Urogen IIIHMDB
Uroporphyrinogen-IIIHMDB
UroporphyrinogensHMDB
Chemical FormulaC40H44N4O16
Average Molecular Weight836.7946
Monoisotopic Molecular Weight836.27523138
IUPAC Name3-[9,14,20-tris(2-carboxyethyl)-5,10,15,19-tetrakis(carboxymethyl)-21,22,23,24-tetraazapentacyclo[16.2.1.1³,⁶.1⁸,¹¹.1¹³,¹⁶]tetracosa-1(20),3,5,8,10,13,15,18-octaen-4-yl]propanoic acid
Traditional Nameuroporphyrinogen-III
CAS Registry Number1976-85-8
SMILES
OC(=O)CCC1=C2CC3=C(CCC(O)=O)C(CC(O)=O)=C(CC4=C(CC(O)=O)C(CCC(O)=O)=C(CC5=C(CC(O)=O)C(CCC(O)=O)=C(CC(N2)=C1CC(O)=O)N5)N4)N3
InChI Identifier
InChI=1S/C40H44N4O16/c45-33(46)5-1-17-21(9-37(53)54)29-14-27-19(3-7-35(49)50)22(10-38(55)56)30(43-27)15-28-20(4-8-36(51)52)24(12-40(59)60)32(44-28)16-31-23(11-39(57)58)18(2-6-34(47)48)26(42-31)13-25(17)41-29/h41-44H,1-16H2,(H,45,46)(H,47,48)(H,49,50)(H,51,52)(H,53,54)(H,55,56)(H,57,58)(H,59,60)
InChI KeyHUHWZXWWOFSFKF-UHFFFAOYSA-N
Chemical Taxonomy
Description belongs to the class of organic compounds known as porphyrins. Porphyrins are compounds containing a fundamental skeleton of four pyrrole nuclei united through the alpha-positions by four methine groups to form a macrocyclic structure.
KingdomOrganic compounds
Super ClassOrganoheterocyclic compounds
ClassTetrapyrroles and derivatives
Sub ClassPorphyrins
Direct ParentPorphyrins
Alternative Parents
Substituents
  • Porphyrin
  • Substituted pyrrole
  • Pyrrole
  • Heteroaromatic compound
  • Carboxylic acid derivative
  • Carboxylic acid
  • Azacycle
  • Carbonyl group
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Organooxygen compound
  • Organonitrogen compound
  • Organic oxygen compound
  • Organic nitrogen compound
  • Aromatic heteropolycyclic compound
Molecular FrameworkAromatic heteropolycyclic compounds
External Descriptors
Ontology
Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

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 Solubility0.044 g/LALOGPS
logP0.67ALOGPS
logP1.39ChemAxon
logS-4.3ALOGPS
pKa (Strongest Acidic)3.21ChemAxon
Physiological Charge-8ChemAxon
Hydrogen Acceptor Count16ChemAxon
Hydrogen Donor Count12ChemAxon
Polar Surface Area361.56 ŲChemAxon
Rotatable Bond Count20ChemAxon
Refractivity206.93 m³·mol⁻¹ChemAxon
Polarizability81.55 ųChemAxon
Number of Rings5ChemAxon
BioavailabilityNoChemAxon
Rule of FiveNoChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-106r-0000000980-8aa242b9a95e3ad92890Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-05fr-0000000910-5ea901953b2295eabfb6Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0ab9-0000000900-fe3587925504317b1a70Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-00xr-0000000930-6a464d08b50b7b5e266cSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-00di-0000000910-22d2fcd94b453076587dSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-00di-2000000900-342f06471315f49e3debSpectrum
Biological Properties
Cellular Locations
  • Cytoplasm
Biospecimen Locations
  • Blood
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodExpected but not QuantifiedNot QuantifiedNot AvailableNot Available
Normal
      Not Available
details
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB031250
KNApSAcK IDC00007373
Chemspider ID1146
KEGG Compound IDC01051
BioCyc IDUROPORPHYRINOGEN-III
BiGG ID36704
Wikipedia LinkUroporphyrinogen III
METLIN ID79
PubChem Compound1179
PDB IDNot Available
ChEBI ID15437
Food Biomarker OntologyNot Available
VMH IDUPPG3
MarkerDB ID
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Aizencang G, Solis C, Bishop DF, Warner C, Desnick RJ: Human uroporphyrinogen-III synthase: genomic organization, alternative promoters, and erythroid-specific expression. Genomics. 2000 Dec 1;70(2):223-31. [PubMed:11112350 ]
  2. Martins BM, Grimm B, Mock HP, Huber R, Messerschmidt A: Crystal structure and substrate binding modeling of the uroporphyrinogen-III decarboxylase from Nicotiana tabacum. Implications for the catalytic mechanism. J Biol Chem. 2001 Nov 23;276(47):44108-16. Epub 2001 Aug 27. [PubMed:11524417 ]

Enzymes

General function:
Involved in uroporphyrinogen decarboxylase activity
Specific function:
Catalyzes the decarboxylation of four acetate groups of uroporphyrinogen-III to yield coproporphyrinogen-III.
Gene Name:
UROD
Uniprot ID:
P06132
Molecular weight:
40786.58
Reactions
Uroporphyrinogen III → Coproporphyrinogen III + CO(2)details
Uroporphyrinogen III → Coproporphyrinogen III + Carbon dioxidedetails
General function:
Involved in uroporphyrinogen-III synthase activity
Specific function:
Catalyzes cyclization of the linear tetrapyrrole, hydroxymethylbilane, to the macrocyclic uroporphyrinogen III, the branch point for the various sub-pathways leading to the wide diversity of porphyrins. Porphyrins act as cofactors for a multitude of enzymes that perform a variety of processes within the cell such as methionine synthesis (vitamin B12) or oxygen transport (heme).
Gene Name:
UROS
Uniprot ID:
P10746
Molecular weight:
28627.37
Reactions
Hydroxymethylbilane → Uroporphyrinogen III + Waterdetails