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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.
- 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.
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