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

delete2026-01-31
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PRE
AI
E
Eunbin Jee
M
Maisha Medha
H
Hwayoung Baek
J
Jonghan Kim
Y
Yuho Kim *
DOI:10.1016/j.mito.2026.102120delete
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Abstract

Abstract

En 中文
• 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.

Journal

MITOCHONDRION cover
MITOCHONDRION
IF:
4.5
Papers:
2.4K
Citations:
5.5K

Organization

U
university of massachusetts
Scholars:
638
Papers: 358
Citations: 0
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