The role of the mitochondrion in cellular iron homeostasis

Nichole D. Schueck, Michael Woontner, David Koeller

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

The yeast ATM1 protein is essential for normal mitochondrial iron homeostasis. Deletion of ATM1 results in mitochondrial iron accumulation and oxidative mitochondrial damage. Mutations in ABC7, the human homolog of ATM1, result in X-linked sideroblastic anemia and ataxia. Here we show that a deletion of ATM1 also has effects on extra-mitochondrial iron metabolism. ATM1-deficient cells have an increased iron requirement for growth. When grown in iron-rich medium, mutant cells accumulate excess mitochondrial iron and have increased expression of the genes required for both high and low affinity iron uptake. Thus, ATM1 mutant cells simultaneously demonstrate features of both iron overload and iron starvation. Yfh1p is the yeast homolog of the human frataxin protein, which is deficient in Friedreich's ataxia. As in atm1 cells, a yfh1 deletion results in both mitochondrial iron accumulation and cytosolic iron starvation. In spite of their apparent roles in cellular iron homeostasis, we find that the expression of neither ATM1 nor YFH1 is responsive to cellular iron status. Based on these observations, we propose a model in which cellular iron is prioritized for use by the mitochondrion, and available to the remainder of the cell only after mitochondrial needs have been fulfilled.

Original languageEnglish (US)
Pages (from-to)51-60
Number of pages10
JournalMitochondrion
Volume1
Issue number1
DOIs
StatePublished - Jun 2001

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Mitochondria
Homeostasis
Iron
Starvation
Friedreich Ataxia
Iron Overload
Fungal Proteins
Yeasts
Gene Expression
Mutation

Keywords

  • Ataxia
  • Iron metabolism
  • Mitochondrion

ASJC Scopus subject areas

  • Biophysics

Cite this

The role of the mitochondrion in cellular iron homeostasis. / Schueck, Nichole D.; Woontner, Michael; Koeller, David.

In: Mitochondrion, Vol. 1, No. 1, 06.2001, p. 51-60.

Research output: Contribution to journalArticle

Schueck, Nichole D. ; Woontner, Michael ; Koeller, David. / The role of the mitochondrion in cellular iron homeostasis. In: Mitochondrion. 2001 ; Vol. 1, No. 1. pp. 51-60.
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