ALA dehydratase (ALAD; porphobilinogen synthase)
Cofactors used
Clinical / pharmacological. Inhibited by lead: lead poisoning with basophilic stippling, elevated ALA
What it does, reaction by reaction
Heme Biosynthesis (Porphyrin Pathway) Amino Acid & Nitrogen Metabolism · Mitochondrion + cytosol
2 ALA → porphobilinogen (PBG) + 2 H₂O
Converts delta-Aminolevulinate (ALA) into Porphobilinogen (PBG) H2O
› Notes
Cytosolic ALA dehydratase (porphobilinogen synthase) condenses two ALA molecules and requires catalytic zinc and sulfhydryl integrity. This is an essentially irreversible condensation; lead inhibits the enzyme by disrupting zinc/sulfhydryl-dependent function.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
Heme Biosynthesis (Porphyrin Pathway)
Listed there as: ALA dehydratase
Adequate zinc and sulfhydryl integrity
Lead; severe zinc deficiency
No major direct hormonal control
Recent literature
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