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| Names | |
|---|---|
| Preferred IUPAC name 3-[5-(Aminomethyl)-4-(carboxymethyl)-1H-pyrrol-3-yl]propanoic acid | |
| Identifiers | |
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3D model (JSmol) | |
| ChemSpider |
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| DrugBank |
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| ECHA InfoCard | 100.006.970 |
| EC Number |
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| MeSH | Porphobilinogen |
| UNII | |
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| Properties | |
| C10H14N2O4 | |
| Molar mass | 226.229 |
Except where otherwise noted, data are given for materials in theirstandard state (at 25 °C [77 °F], 100 kPa). | |
Porphobilinogen (PBG) is anorganic compound that occurs in living organisms as an intermediate in the biosynthesis ofporphyrins, which include critical substances likehemoglobin andchlorophyll.[1]
The structure of the molecule can be described as molecule ofpyrrole with sidechains substituted forhydrogen atoms at positions 2, 3 and 4 in the ring (1 being thenitrogen atom); respectively, anaminomethyl group−CH2−NH2, anacetic acid (carboxymethyl) group−CH2−COOH, and apropionic acid (carboxyethyl) group−CH2−CH2−COOH.
In the first step of theporphyrin biosynthesis pathway, porphobilinogen is generated fromaminolevulinate (ALA) by theenzymeALA dehydratase.
In the typical porphyrin biosynthesis pathway, four molecules of porphobilinogen are concatenated by carbons 2 and 5 of the pyrrole ring (adjacent to thenitrogen atom) intohydroxymethyl bilane by the enzymeporphobilinogen deaminase, also known ashydroxymethylbilane synthase.
Acute intermittent porphyria causes an increase in urinary porphobilinogen.[2]
