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Heme biosynthesis pathway showing chemical intermediates and sites of production (mitochondria versus cytosol)

Heme biosynthesis pathway showing chemical intermediates and sites of production (mitochondria versus cytosol)
Heme synthesis begins with the formation of delta-aminolevulinic acid (ALA) from glycine and succinyl-CoA by ALA synthase (ALAS); this step is rate-limiting in the liver. Hepatic ALAS is mainly regulated by heme via feedback repression (dashed arrow at the top of the frame). Refer to UpToDate for a discussion of porphyrias that result from deficiencies of these enzymes.
CoA: coenzyme A.
Reproduced from: Anderson KE. The porphyrias. In: Zakim and Boyer's Hepatology: A Textbook of Liver Disease, 5th ed, Boyer TD, Wright TL, Manns MP, Zakim D (Eds), Elsevier, Philadelphia 2006. Illustration used with the permission of Elsevier Inc. All rights reserved.
Graphic 117995 Version 1.0