Article

Pharmacological activation of AMPK rescues mitophagy defects in models of FBXL4-related mitochondrial DNA depletion syndrome

2025-12-03

Abstract excerpt

Distinct mitophagy pathways can eliminate not only damaged mitochondria but also healthy ones. In Mitochondrial DNA Depletion Syndrome 13 (MTDPS13), dysregulated BNIP3/NIX-driven mitophagy of functional mitochondria is thought to be the key pathological driver. Patient mutations in the E3 ubiquitin ligase FBXL4 impair the proteasomal degradation of the mitophagy receptors BNIP3 and NIX, causing their accumulation...

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Literature Corpus work: 7e7e9762-f467-5601-9a06-b215f7996a32
DOI: 10.64898/2025.12.03.692069

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