Porphobilinogen (BioCAD00000016531)

blood urine erythrocyte liver plant natural products

Metabolite Card

Chinese Name: 原卟啉原
Formula: C10H14N2O4 (226.0954)
SMILES:

NCC1=C(CC(O)=O)C(CCC(O)=O)=CN1

Synonyms [en]

Porphobilinogen; 3-[5-(AMINOMETHYL)-4-(CARBOXYMETHYL)-1H-PYRROL-3-YL]PROPANOIC ACID; 5-(Aminomethyl)-4-(carboxymethyl)-pyrrole-3-propionate; PBG; 2-aminomethylpyrrol-3-acetic acid 4-propionic acid; 5-(Aminomethyl)-4-(carboxymethyl)-1H-pyrrole-3-propanoic acid

Reviewed

Last reviewed on 2026-04-16.

Cite this Page

Porphobilinogen. 数据之源,洞见之始. SMRUCC genomics institute, a synthetic life researcher from China. https://biocad_registry.innovation.ac.cn/s/Porphobilinogen (retrieved 2026-08-21) (CAD Registry RN: BioCAD00000016531). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

Note

Porphobilinogen (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).

Entity Information

DBLinks

Other DBLinks
  • CAS Registry Number: 487-90-1
  • PubChem: 1021
  • ChEBI: ChEBI:17381
  • HMDB: HMDB0000245
  • KEGG: C00931
  • BioCyc: PORPHOBILINOGEN
  • NCBI MeSH: Porphobilinogen
  • Wikipedia: Porphobilinogen
  • DrugBank: DB02272
  • RefMet: RM0138927
  • MoNA: BAF_UVA_POS000605
  • MoNA: BAF_UVA_POS001350
  • MoNA: BAF_UVA_POS001351
  • MoNA: BAF_UVA_POS001352
  • MoNA: EMBL_MCF_2_0_HRMS_Library000240
  • MoNA: FiehnHILIC001461
  • MoNA: FiehnHILIC002269
  • MoNA: FiehnHILIC002940
  • MoNA: HMDB0000245_c_ms_1965
  • MoNA: HMDB0000245_ms_ms_415
  • MoNA: HMDB0000245_ms_ms_416
  • MoNA: HMDB0000245_ms_ms_417
  • Metlin: METLIN_76
  • Coconut NaturalProduct: CNP0307889.0
  • PMHub: MS000000949
  • metaboanalyst: 5e533b1268fd0c54bbb449bab2a75fc9
  • metaboanalyst: b1a35c068006465ba46e493b21296941

Class / Ontology

Metabolic Network
ID EC Number Name
KEGG:R00036 4.2.1.24 5-aminolevulinate hydro-lyase (adding 5-aminolevulinate and cyclizing; porphobilinogen-forming)
KEGG:R00084 2.5.1.61 porphobilinogen:(4-[2-carboxyethyl]-3-[carboxymethyl]pyrrol-2-yl)methyltransferase (hydrolysing);
BioCyc:RXN-21523 Hydroxymethylbilane-Synthase-ES2 + PORPHOBILINOGEN --> Hydroxymethylbilane-Synthase-ES3 + AMMONIUM
BioCyc:PORPHOBILSYNTH-RXN 4.2.1.24 2 5-AMINO-LEVULINATE --> PROTON + 2 WATER + PORPHOBILINOGEN
BioCyc:OHMETHYLBILANESYN-RXN 2.5.1.61 WATER + 4 PORPHOBILINOGEN --> 4 AMMONIUM + HYDROXYMETHYLBILANE
BioCyc:RXN-21527 Hydroxymethylbilane-Synthase-ES + PORPHOBILINOGEN --> Hydroxymethylbilane-Synthase-ES2 + AMMONIUM
BioCyc:RXN-21526 Holo-Hydroxymethylbilane-Synthase + PORPHOBILINOGEN --> Hydroxymethylbilane-Synthase-ES + AMMONIUM
BioCyc:RXN-21524 Hydroxymethylbilane-Synthase-ES3 + PORPHOBILINOGEN --> Hydroxymethylbilane-Synthase-ES4 + AMMONIUM
View More
Mass Spectrum
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View All Mass Spectrum
Organism Source

Taxonomy Source

  1. Arabidopsis thaliana [ncbi taxid: 3702]
  2. Citrullus lanatus [ncbi taxid: 3654]
  3. Escherichia coli [ncbi taxid: 562]
  4. Homo sapiens [ncbi taxid: 9606]
  5. Mus musculus [ncbi taxid: 10090]
  6. Saccharomyces cerevisiae [ncbi taxid: 4932]

Pathway Synthetic

pathway id name
BioCyc:CALBI_PWY3B3-2 tetrapyrrole biosynthesis
PathBank:SMP0000953 Porphyrin Metabolism
WikiPathways:WP86 Heme biosynthesis
WikiPathways:WP1221 Heme biosynthesis
WikiPathways:WP3604 Biochemical pathways: part I
WikiPathways:WP1314 Heme biosynthesis
WikiPathways:WP5169 Hemesynthesis defects and porphyrias
WikiPathways:WP848 Heme biosynthesis
WikiPathways:WP967 Heme biosynthesis
WikiPathways:WP561 Heme biosynthesis
WikiPathways:WP1086 Heme biosynthesis
WikiPathways:WP102 Heme biosynthesis
WikiPathways:WP4506 Tyrosine metabolism and related disorders
PathBank:SMP0120492 Hereditary Coproporphyria (HCP)
PathBank:SMP0120544 Porphyria Variegata (PV)
PathBank:SMP0000024 Porphyrin Metabolism
PathBank:SMP0120689 Congenital Erythropoietic Porphyria (CEP) or Gunther Disease
PathBank:SMP0120447 Acute Intermittent Porphyria
PathBank:SMP0000345 Congenital Erythropoietic Porphyria (CEP) or Gunther Disease
PathBank:SMP0087397 Porphyrin Metabolism
View All Pathways