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Article

The ins and outs of mitochondrial iron-loading: the metabolic defect in Friedreich's ataxia.

Journal of molecular medicine (Berlin, Germany) - 1 Apr 2010

Richardson Des R, Huang Michael L-H, Whitnall Megan, Becker Erika M, Ponka Prem, Suryo Rahmanto Yohan

Abstract excerpt

Friedreich's ataxia is a cardio- and neurodegenerative disease due to decreased expression of the mitochondrial protein, frataxin. This defect results in mitochondrial iron-overload, and in this review, we discuss the mechanisms that lead to this iron accumulation. Using a conditional knockout mouse model where frataxin is deleted in the heart, it has been shown that this mutation leads to transferrin receptor-1...

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Identifiers

PMID: 19997898
DOI: 10.1007/s00109-009-0565-x

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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