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Record Information
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-03-05 03:06:01 UTC
Secondary Accession Numbers
  • HMDB00245
Metabolite Identification
Common NamePorphobilinogen
DescriptionPorphobilinogen (PBG) is a pyrrole-containing intermediate in the biosynthesis of porphyrins. It is generated from aminolevulinate (ALA) by the enzyme ALA dehydratase. Porphobilinogen is then converted into hydroxymethylbilane by the enzyme porphobilinogen deaminase (also known as hydroxymethylbilane synthase). Under certain conditions, porphobilinogen can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, and hereditary coproporphyria (HCP). There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503 ).
5-(Aminomethyl)-4-(carboxymethyl)-pyrrole-3-propionic acidHMDB
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
InChI Identifier
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 ClassOrganic nitrogen compounds
ClassOrganonitrogen compounds
Sub ClassAmines
Direct ParentAralkylamines
Alternative Parents
  • Aralkylamine
  • Dicarboxylic acid or derivatives
  • Substituted pyrrole
  • Heteroaromatic compound
  • Pyrrole
  • Amino acid
  • Amino acid or derivatives
  • Carboxylic acid derivative
  • Azacycle
  • Carboxylic acid
  • Organoheterocyclic compound
  • Organopnictogen compound
  • Primary amine
  • Organooxygen compound
  • Primary aliphatic amine
  • Organic oxygen compound
  • Hydrocarbon derivative
  • Carbonyl group
  • Organic oxide
  • Aromatic heteromonocyclic compound
Molecular FrameworkAromatic heteromonocyclic compounds
External Descriptors
Physiological effect

Health effect:

  Health condition:


Biological location:


  Biofluid and excreta:

  Organ and components:


Route of exposure:





Naturally occurring process:

  Biological process:

    Biochemical pathway:


Industrial application:

  Pharmaceutical industry:

Biological role:

Physical Properties
Experimental Properties
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility2.72 g/LALOGPS
pKa (Strongest Acidic)3.66ChemAxon
pKa (Strongest Basic)8.99ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count4ChemAxon
Polar Surface Area116.41 ŲChemAxon
Rotatable Bond Count6ChemAxon
Refractivity56.38 m³·mol⁻¹ChemAxon
Polarizability22.53 ųChemAxon
Number of Rings1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-MS (4 TMS)splash10-001j-1923000000-70c31b91868484322655View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-001j-1923000000-70c31b91868484322655View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-001i-3920000000-d4006159f28f6672d3a5View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-0080-9071000000-ad88926555e7416fedb3View in MoNA
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
Acute Intermittent PorphyriaThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Congenital Erythropoietic Porphyria (CEP) or Gunther DiseaseThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Hereditary Coproporphyria (HCP)ThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Porphyria Variegata (PV)ThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Porphyrin MetabolismThumbThumb?image type=greyscaleThumb?image type=simpleMap00860
Normal Concentrations
BloodDetected and Quantified0.06 (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...
UrineDetected and Quantified0.29 +/- 0.14 umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
UrineDetected and Quantified0-1.5 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified<=0.579 umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BloodDetected and Quantified3.1 +/- 1.0 uMAdult (>18 years old)Both
UrineDetected and Quantified57.1 (19.6-132.7) umol/mmol creatinineAdult (>18 years old)Both
Acute intermittent porphyria
UrineDetected and Quantified17.2 (74.9-41.6) umol/mmol creatinineAdult (>18 years old)Both
Acute intermittent porphyria
UrineDetected and Quantified9.7 (21.8-10.5) umol/mmol creatinineAdult (>18 years old)Both
Acute intermittent porphyria
UrineDetected and Quantified2.3 (5.1-2.9) umol/mmol creatinineAdult (>18 years old)Both
Asymptomatic acute intermittent porphyria
UrineDetected and Quantified0.589-0.654 umol/mmol creatinineAdult (>18 years old)MaleProtoporphyria, Erythropoietic details
Associated Disorders and Diseases
Disease References
Acute intermittent porphyria
  1. Marsden JT, Rees DC: Urinary excretion of porphyrins, porphobilinogen and delta-aminolaevulinic acid following an attack of acute intermittent porphyria. J Clin Pathol. 2014 Jan;67(1):60-5. doi: 10.1136/jclinpath-2012-201367. Epub 2013 Aug 1. [PubMed:23908454 ]
  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. [PubMed:16497943 ]
Protoporphyria, Erythropoietic
  1. MAGNUS IA, JARRETT A, PRANKERD TA, RIMINGTON C: Erythropoietic protoporphyria. A new porphyria syndrome with solar urticaria due to protoporphyrinaemia. Lancet. 1961 Aug 26;2(7200):448-51. [PubMed:13765301 ]
Associated OMIM IDs
  • 176000 (Acute intermittent porphyria)
  • 177000 (Protoporphyria, Erythropoietic)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB021916
KNApSAcK IDC00007339
Chemspider ID995
KEGG Compound IDC00931
BiGG ID36404
Wikipedia LinkPorphobilinogen
PubChem Compound1021
ChEBI ID17381
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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed:7258864 ]
  12. Winkelman JW, Collins GH: Neurotoxicity of tetraphenylporphinesulfonate TPPS4 and its relation to photodynamic therapy. Photochem Photobiol. 1987 Nov;46(5):801-7. [PubMed:3441503 ]


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:
Uniprot ID:
Molecular weight:
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:
Uniprot ID:
Molecular weight:
5-Aminolevulinic acid → Porphobilinogen + Waterdetails