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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:02:22 UTC
HMDB IDHMDB00245
Secondary Accession NumbersNone
Metabolite Identification
Common NamePorphobilinogen
DescriptionPorphobilinogen is a pyrrole involved in porphyrin metabolism. -- Wikipedia; It consists of a pyrrole ring with acetyl, propionyl, and aminomethyl side chains; It is a key monopyrrolic intermediate in porphyrin, chlorophyll and vitamin B12 biosynthesis. Porphobilinogen is generated by the enzyme ALA dehydratase by combining two molecules of dALA together, and converted into hydroxymethyl bilane by the enzyme porphobilinogen deaminase. 4 molecules of porphobilinogen are condensed to form one molecule of uroporphyrinogen III, which is then converted successively to coproporphyrinogen III, protoporphyrin IX, and heme. Porphobilinogen is produced in excess and excreted in the urine in acute intermittent porphyria and several other porphyrias.
Structure
Thumb
Synonyms
ValueSource
5-(Aminomethyl)-4-(carboxymethyl)-pyrrole-3-propionateHMDB
5-(Aminomethyl)-4-(carboxymethyl)-pyrrole-3-propionic acidHMDB
PBGHMDB
Chemical FormulaC10H14N2O4
Average Molecular Weight226.2292
Monoisotopic Molecular Weight226.095356946
IUPAC Name3-[5-(aminomethyl)-4-(carboxymethyl)-1H-pyrrol-3-yl]propanoic acid
Traditional Nameporphobilinogen
CAS Registry Number487-90-1
SMILES
NCC1=C(CC(O)=O)C(CCC(O)=O)=CN1
InChI Identifier
InChI=1S/C10H14N2O4/c11-4-8-7(3-10(15)16)6(5-12-8)1-2-9(13)14/h5,12H,1-4,11H2,(H,13,14)(H,15,16)
InChI KeyInChIKey=QSHWIQZFGQKFMA-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as aralkylamines. These are alkylamines in which the alkyl group is substituted at one carbon atom by an aromatic hydrocarbyl group.
KingdomOrganic compounds
Super ClassOrganonitrogen compounds
ClassAmines
Sub ClassAralkylamines
Direct ParentAralkylamines
Alternative Parents
Substituents
  • Aralkylamine
  • Substituted pyrrole
  • Dicarboxylic acid or derivatives
  • Heteroaromatic compound
  • Pyrrole
  • Azacycle
  • Organoheterocyclic compound
  • Carboxylic acid
  • Carboxylic acid derivative
  • Hydrocarbon derivative
  • Primary amine
  • Organooxygen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Aromatic heteromonocyclic compound
Molecular FrameworkAromatic heteromonocyclic compounds
External Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
Biofunction
  • Component of Porphyrin and chlorophyll metabolism
ApplicationNot Available
Cellular locations
  • Cytoplasm (predicted from logP)
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 Solubility2.72 mg/mLALOGPS
logP-2.4ALOGPS
logP-2.7ChemAxon
logS-1.9ALOGPS
pKa (Strongest Acidic)3.66ChemAxon
pKa (Strongest Basic)8.99ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count4ChemAxon
Polar Surface Area116.41 Å2ChemAxon
Rotatable Bond Count6ChemAxon
Refractivity56.38 m3·mol-1ChemAxon
Polarizability22.53 Å3ChemAxon
Number of Rings1ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-MS (4 TMS)splash10-001j-1923000000-70c31b91868484322655View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MSNot Available
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0a4i-0090000000-912aa953bdd13d6d18caView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00dl-1900000000-8a87960bb63c4915db22View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-006x-9500000000-c934217374e64d6edee3View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0a4l-0980000000-423d28b762ba4c07586bView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-03dl-0910000000-31575764ba8bd8d50279View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-074r-0900000000-9d96bd59f1a4371a8df4View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0059-0790000000-2cc911a67c1aa8e35d99View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-06ur-0940000000-2c3da60da6ab9523fbf3View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0bt9-4900000000-cf52dffb4284f9eeaa5dView in MoNA
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Blood
  • Urine
Tissue Location
  • Erythrocyte
  • Liver
Pathways
NameSMPDB LinkKEGG Link
Acute Intermittent PorphyriaSMP00344Not Available
Congenital Erythropoietic Porphyria (CEP) or Gunther DiseaseSMP00345Not Available
Hereditary Coproporphyria (HCP)SMP00342Not Available
Porphyria Variegata (PV)SMP00346Not Available
Porphyrin MetabolismSMP00024map00860
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified 0.06 (0.00-0.12) uMAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.31 +/- 0.13 umol/mmol creatinineChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified0.29 +/- 0.14 umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified<=0.579 umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified3.1 +/- 1.0 uMAdult (>18 years old)Both
Porphyria
details
Associated Disorders and Diseases
Disease References
Porphyria
  1. Floderus Y, Sardh E, Moller C, Andersson C, Rejkjaer L, Andersson DE, Harper P: Variations in porphobilinogen and 5-aminolevulinic acid concentrations in plasma and urine from asymptomatic carriers of the acute intermittent porphyria gene with increased porphyrin precursor excretion. Clin Chem. 2006 Apr;52(4):701-7. Epub 2006 Feb 23. [16497943 ]
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB021916
KNApSAcK IDNot Available
Chemspider ID995
KEGG Compound IDC00931
BioCyc IDPORPHOBILINOGEN
BiGG ID36404
Wikipedia LinkPorphobilinogen
NuGOwiki LinkHMDB00245
Metagene LinkHMDB00245
METLIN ID76
PubChem Compound1021
PDB IDPBG
ChEBI ID17381
References
Synthesis ReferenceFrydman, Benjamin; Despuy, Maria E.; Rapoport, Henry. Pyrroles from azaindoles. A synthesis of porphobilinogen. Journal of the American Chemical Society (1965), 87(15), 3530-1.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Dhar GJ, Bossenmaier I, Petryka ZJ, Cardinal R, Watson CJ: Effects of hematin in hepatic porphyria. Further studies. Ann Intern Med. 1975 Jul;83(1):20-30. [1147435 ]
  2. Ivanov E, Pisanets M: Studies on the biosynthesis of porphyrins in erythrocytes after incubation with delta-aminolevulinic acid: an attempt to investigate the pathogenesis of nephrogenic anemia. Acta Biol Med Ger. 1982;41(4):307-13. [7124248 ]
  3. Ellencweig N, Schoenfeld N, Zemishlany Z: Acute intermittent porphyria: psychosis as the only clinical manifestation. Isr J Psychiatry Relat Sci. 2006;43(1):52-6. [16910386 ]
  4. Buchet JP, Lauwerys R, Hassoun A, Dratwa M, Wens R, Collart F, Tielemans C: Effect of aluminum on porphyrin metabolism in hemodialyzed patients. Nephron. 1987;46(4):360-3. [3658064 ]
  5. Tishler PV, Woodward B, O'Connor J, Holbrook DA, Seidman LJ, Hallett M, Knighton DJ: High prevalence of intermittent acute porphyria in a psychiatric patient population. Am J Psychiatry. 1985 Dec;142(12):1430-6. [4073306 ]
  6. Hsiao KJ, Lee FY, Wu SJ, Chang WJ: Determination of erythrocyte porphobilinogen deaminase activity using porphobilinogen as substrate. Clin Chim Acta. 1987 Sep 30;168(2):257-8. [3677422 ]
  7. Evans J, Lefkowitch J, Lim CK, Billing B: Fecal porphyrin abnormalities in a patient with features of Rotor's syndrome. Gastroenterology. 1981 Dec;81(6):1125-30. [7286590 ]
  8. Sassa S, Solish G, Levere RD, Kappas A: Studies in porphyria. IV. Expression of the gene defect of acute intermittent porphyria in cultured human skin fibroblasts and amniotic cells: prenatal diagnosis of the porphyric trait. J Exp Med. 1975 Sep 1;142(3):722-31. [1165472 ]
  9. Ford RE, Ou CN, Ellefson RD: Assay for erythrocyte uroporphyrinogen I synthase activity, with porphobilinogen as substrate. Clin Chem. 1980 Jul;26(8):1182-5. [7389090 ]
  10. Shiue JW, Lee FY, Hsiao KJ, Tsai YT, Lee SD, Wu SJ: Abnormal thyroid function and hypercholesterolemia in a case of acute intermittent porphyria. Taiwan Yi Xue Hui Za Zhi. 1989 Jul;88(7):729-31. [2809566 ]
  11. Mustajoki P: Normal erythrocyte uroporphyrinogen I synthase in a kindred with acute intermittent porphyria. Ann Intern Med. 1981 Aug;95(2):162-6. [7258864 ]

Enzymes

General function:
Involved in hydroxymethylbilane synthase activity
Specific function:
Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
Gene Name:
HMBS
Uniprot ID:
P08397
Molecular weight:
39329.74
Reactions
Porphobilinogen + Water → Hydroxymethylbilane + Ammoniadetails
General function:
Involved in porphobilinogen synthase activity
Specific function:
Catalyzes an early step in the biosynthesis of tetrapyrroles. Binds two molecules of 5-aminolevulinate per subunit, each at a distinct site, and catalyzes their condensation to form porphobilinogen.
Gene Name:
ALAD
Uniprot ID:
P13716
Molecular weight:
36294.485
Reactions
5-Aminolevulinic acid → Porphobilinogen + Waterdetails