The Heme Biosynthesis Pathway and Clinical Manifestations of Abnormal Function

John D. Phillips1, James P. Kushner1

1 University of Utah Medical School, Salt Lake City, Utah
Publication Name:  Current Protocols in Toxicology
Unit Number:  Unit 8.1
DOI:  10.1002/0471140856.tx0801s00
Online Posting Date:  May, 2001
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Biosynthesis of heme is important for both prokaryotes and eukaryotes. The enzymes for this multistep process are distributed between the cytosol and mitochondria in eukaryotes. In humans there are inherited and acquired disorders characterized by over synthesis of one or more enzymes or absence of an enzyme. This overview discusses each enzyme in the heme biosynthesis pathway.

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Table of Contents

  • ALA Synthase (ALAS)
  • ALA Dehydratase (ALAD)
  • Porphobilinogen Deaminase (PBGD)
  • Uroporphyrinogen III Cosynthase (UCoS)
  • Uroporphyrinogen Decarboxylase (UROD)
  • Coproporphyrinogen Oxidase (CPO)
  • Protoporphyrinogen Oxidase (PPO)
  • Ferrochelatase
  • Interpretation of Urinary Porphyrin Excretion
  • Summary
  • Figures
  • Tables
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Literature Cited

Literature Cited
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