The mitochondrial heme metabolon: Insights into the complex(ity) of heme synthesis and distribution

Robert B. Piel, Harry A. Dailey, Amy E. Medlock

Research output: Contribution to journalReview article

2 Scopus citations

Abstract

Heme is an essential cofactor in metazoans that is also toxic in its free state. Heme is synthesized by most metazoans and must be delivered to all cellular compartments for incorporation into a variety of hemoproteins. The heme biosynthesis enzymes have been proposed to exist in a metabolon, a protein complex consisting of interacting enzymes in a metabolic pathway. Metabolons enhance the function of enzymatic pathways by creating favorable microenvironments for pathway enzymes and intermediates, facilitating substrate transport, and providing a scaffold for interactions with other pathways, signaling molecules, or organelles. Herein we detail growing evidence for a mitochondrial heme metabolon and discuss its implications for the study of heme biosynthesis and cellular heme homeostasis.

Original languageEnglish (US)
Pages (from-to)198-203
Number of pages6
JournalMolecular Genetics and Metabolism
Volume128
Issue number3
DOIs
StatePublished - Nov 2019

Keywords

  • Anemia
  • Heme
  • Heme Biosynthesis
  • Metabolon
  • Porphyria

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Biochemistry
  • Molecular Biology
  • Genetics
  • Endocrinology

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