Porphobilinogen (BioCAD00000016531)
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
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).
DBLinks
- CAS Registry Number: 487-90-1
- PubChem CID: 1021
- ChEBI: 17381
- HMDB: HMDB0000245
- LipidMaps:
- KEGG: C00931
- BioCyc: PORPHOBILINOGEN
- NCBI MeSH: Porphobilinogen
- Wikipedia: Porphobilinogen
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
- WishartLab ClassyFire: [Amines] Amines
- RefMet: [Aralkylamines] Aralkylamines
- ChEBI: [CHEBI:17381] porphobilinogen
- Coconut NaturalProduct: [Aminoacids] Aminoacids
| 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 |
Taxonomy Source
- Arabidopsis thaliana [ncbi taxid: 3702]
- Citrullus lanatus [ncbi taxid: 3654]
- Escherichia coli [ncbi taxid: 562]
- Homo sapiens [ncbi taxid: 9606]
- Mus musculus [ncbi taxid: 10090]
- 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 |