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©2013 UpToDate ® Print Email Enzyme defect in ALA dehydratase porphyria Depiction of the enzymatic defect in 5-aminolevulinic acid (ALA) dehydratase porphyria (ADP). This disorder is characterized by a defect in the enzyme ALA dehydratase (ALAD), leading to the accumulation and excretion of ALA. The reduced production of heme also contributes via loss of heme-mediated repression of ALA synthase (ALAS), the enzyme that promotes the formation of ALA from glycine and succinyl CoA.