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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-10-09 21:08:42 UTC
HMDB IDHMDB0000245
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 ).
Structure
Thumb
Synonyms
ValueSource
5-(Aminomethyl)-4-(carboxymethyl)-pyrrole-3-propionateHMDB
5-(Aminomethyl)-4-(carboxymethyl)-pyrrole-3-propionic acidHMDB
PBGHMDB
PorphobilinogenHMDB
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 KeyQSHWIQZFGQKFMA-UHFFFAOYSA-N
Chemical Taxonomy
Description 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
Substituents
  • 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
Ontology
Physiological effect
Disposition
Biological locationRoute of exposureSource
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-MetCCS_train_neg146.21730932474
[M-H]-Not Available146.217http://allccs.zhulab.cn/database/detail?ID=AllCCS00000423
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Urine
Tissue Locations
  • Erythrocyte
  • Liver
Pathways
Normal Concentrations
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified3.1 +/- 1.0 uMAdult (>18 years old)Both
Porphyria
details
UrineDetected and Quantified57.1 (19.6-132.7) umol/mmol creatinineAdult (>18 years old)Both
Acute intermittent porphyria
details
UrineDetected and Quantified17.2 (74.9-41.6) umol/mmol creatinineAdult (>18 years old)Both
Acute intermittent porphyria
details
UrineDetected and Quantified9.7 (21.8-10.5) umol/mmol creatinineAdult (>18 years old)Both
Acute intermittent porphyria
details
UrineDetected and Quantified2.3 (5.1-2.9) umol/mmol creatinineAdult (>18 years old)Both
Asymptomatic acute intermittent porphyria
details
UrineDetected and Quantified0.589-0.654 umol/mmol creatinineAdult (>18 years old)MaleProtoporphyria, Erythropoietic 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. [PubMed:16497943 ]
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 ]
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 IDDB02272
Phenol Explorer Compound IDNot Available
FooDB IDFDB021916
KNApSAcK IDC00007339
Chemspider ID995
KEGG Compound IDC00931
BioCyc IDPORPHOBILINOGEN
BiGG IDNot Available
Wikipedia LinkPorphobilinogen
METLIN IDNot Available
PubChem Compound1021
PDB IDNot Available
ChEBI ID17381
Food Biomarker OntologyNot Available
VMH IDPPBNG
MarkerDB IDMDB00013424
Good Scents IDNot Available
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)Not Available
General References

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