Porphobilinogen

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An intermediary product in the biosynthesis of heme. Not detectable in normal animals.

• p. deaminase — an enzyme involved in the condensation of porphobilinogen. Now called uroporphyrinogen I synthetase.

Porphobilinogen
IUPAC name 3-[5-(Aminomethyl)-4-(carboxymethyl)-1H-pyrrol-3-yl]propanoic acid
Identifiers
CAS number	487-90-1
PubChem	1021
EC number	207-666-3
DrugBank	DB02272
MeSH	Porphobilinogen
Jmol-3D images	Image 1
SMILES C1=C(C(=C(N1)CN)CC(=O)O)CCC(=O)O
Properties
Molecular formula	C10H14N2O4
Molar mass	226.229
Y (verify) (what is: Y/N?) Except where noted otherwise, data are given for materials in their standard state (at 25 °C, 100 kPa)
Infobox references

Porphobilinogen (PBG) is a pyrrole involved in porphyrin metabolism.

It is generated by aminolevulinate (ALA) and the enzyme ALA dehydratase. PBG is then converted into hydroxymethyl bilane by the enzyme porphobilinogen deaminase, also known as hydroxymethylbilane synthase.

Acute intermittent porphyria causes an increase in urinary porphobilinogen.^[1]

References

1. ^ Aarsand, AK; Petersen PH, Sandberg S (April 2006). "Estimation and application of biological variation of urinary delta-aminolevulinic acid and porphobilinogen in healthy individuals and in patients with acute intermittent porphyria". Clinical Chemistry 52 (4): 650–656. doi:10.1373/clinchem.2005.060772. PMID 16595824. http://www.clinchem.org/cgi/content/full/52/4/650. 

v t e Heme metabolic intermediates Porphyrin biosynthesis early mitochondrial: D-Aminolevulinic acid cytosolic: Porphobilinogen · Hydroxymethylbilane · Uroporphyrinogen III · Coproporphyrinogen III late mitochondrial: Protoporphyrinogen IX · Protoporphyrin IX Heme degradation and excretion Breakdown of heme spleen: Heme → Biliverdin → Bilirubin blood: Bilirubin+Albumin liver: Bilirubin diglucuronide Intestine, excretion in feces Stercobilinogen · Stercobilin · Urobilinogen Kidney, excretion in urine Urobilin M: MET mt, k, c/g/r/p/y/i, f/h/s/l/o/e, a/u, n, m k, cgrp/y/i, f/h/s/l/o/e, au, n, m, epon m(A16/C10),i(k, c/g/r/p/y/i, f/h/s/o/e, a/u, n, m) M: MYL cell/phys (coag, heme, immu, gran), csfs rbmg/mogr/tumr/hist, sysi/epon, btst drug (B1/2/3+5+6), btst, trns biochemical families: proteins (amino acids/intermediates) · nucleic acids (constituents/intermediates) · carbohydrates (glycoproteins, alcohols, glycosides) lipids (fatty acids/intermediates, phospholipids, steroids, sphingolipids, eicosanoids) · tetrapyrroles/intermediates

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