4328 Porphobilinogen Porphobilinogen is a pyrrole involved in porphyrin metabolism. 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. 487-90-1 1021 C10H14N2O4 White powder. No indication of carcinogenicity to humans (not listed by IARC). This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation. Chronically high levels of porophyrins are associated with porphyrias such as Porphyria variegate, Acute Intermittent Porphyria and Hereditary Coproporphyria (HCP). 2014-08-29T06:11:46Z 2026-05-14T17:33:00Z Porphobilinogen C00931 17381 PORPHOBILINOGEN DB02272 PBG true NCC1=C(CC(O)=O)C(CCC(O)=O)=CN1 C10H14N2O4 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) QSHWIQZFGQKFMA-UHFFFAOYSA-N 226.2292 226.095356946 Endogenous Solid -2.7 HMDB00245 995 CHEM003234 DTXSID4060070 NS00015147 3-[5-(aminomethyl)-4-(carboxymethyl)-1H-pyrrol-3-yl]propanoic acid 116.41 56.3839 22.557878019255046 6 5 4 3.6647253376201103 8.782950994059014 -1 1 -2.40 -1.92 2.72e+00 g/l