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Article

Frataxin deficiency causes upregulation of mitochondrial Lon and ClpP proteases and severe loss of mitochondrial Fe-S proteins.

The FEBS journal - 1 Feb 2009

Guillon Blanche, Bulteau Anne-Laure, Wattenhofer-Donzé Marie, Schmucker Stéphane, Friguet Bertrand, Puccio Hélène, Drapier Jean-Claude, Bouton Cécile

Abstract excerpt

Friedreich ataxia (FRDA) is a rare hereditary neurodegenerative disease characterized by progressive ataxia and cardiomyopathy. The cause of the disease is a defect in mitochondrial frataxin, an iron chaperone involved in the maturation of Fe-S cluster proteins. Several human diseases, including cardiomyopathies, have been found to result from deficiencies in the activity of specific proteases, which have...

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Identifiers

PMID: 19154341
DOI: 10.1111/j.1742-4658.2008.06847.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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