Mitochondrial iron metabolism and its role in diseases

Clin Chim Acta. 2021 Feb;513:6-12. doi: 10.1016/j.cca.2020.12.005. Epub 2020 Dec 9.


Iron is one of the most important elements for life, but excess iron is toxic. Intracellularly, mitochondria are the center of iron utilization requiring sufficient amounts to maintain normal physiologic function. Accordingly, disruption of iron homeostasis could seriously impact mitochondrial function leading to impaired energy state and potential disease development. In this review, we discuss mechanisms of iron metabolism including transport, processing, heme synthesis, iron-sulfur cluster biogenesis and storage. We highlight the vital role of mitochondrial iron in pathologic states including neurodegenerative disorders and sideroblastic anemia.

Keywords: Heme synthesis; Iron-sulfur cluster biogenesis; Mitochondrial iron; Neurodegenerative disorders; Sideroblastic anemia.

Publication types

  • Review

MeSH terms

  • Anemia, Sideroblastic*
  • Homeostasis
  • Humans
  • Iron
  • Iron Overload*
  • Mitochondria


  • Iron