Mitochondrial iron overload is associated with lysosomal dysfunction-mediated mitophagy impairment in the heart of Friedreich’s ataxia

Eunbin Jee

doi:10.1016/j.mito.2026.102120

Mitochondrial iron overload is associated with lysosomal dysfunction-mediated mitophagy impairment in the heart of Friedreich’s ataxia

2026-01-31

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AI

E

Eunbin Jee

M

Maisha Medha

H

Hwayoung Baek

J

Jonghan Kim

Y

Yuho Kim *

DOI：10.1016/j.mito.2026.102120

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• Mitochondrial iron deposits are associated with altered mitochondrial ultrastructure (cristae) and impaired mitochondrial function under cardiac frataxin deficiency, which may lead to Friedreich’s ataxia-related cardiomyopathy. • Dysfunctional lysosomal capacity may contribute to impaired clearance of dysfunctional mitochondria in the context of cardiac-specific frataxin deficiency.

Mitochondrial iron overload is associated with lysosomal dysfunction-mediated mitophagy impairment in the heart of Friedreich’s ataxia

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