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Targeting mitochondrial deubiquitinase USP30 to induce mitophagy in heteroplasmic mitochondrial diseases.

Pharmacological reports : PR - 1 Apr 2026

Pinho Brígida R, Martins Vasco, Chacko Anitta R, Nogueira Célia, Duchen Michael R, Oliveira Jorge M A

Abstract excerpt

BACKGROUND: Mitochondrial DNA (mtDNA) diseases are heterogeneous and lack effective treatments. Their severity correlates with mutant mtDNA load. Mitophagy degrades dysfunctional mitochondria, contributing to a healthy mitochondrial pool. USP30, a mitochondrial deubiquitinase, limits mitophagy by removing the ubiquitin tagging mitochondria for degradation. We investigated whether inhibiting USP30 could enhance...

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Benzoates
Furans
Mitochondrial Diseases
Mitophagy
Oxidative Phosphorylation

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Thiolester Hydrolases

Ubiquitin Thiolesterase

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Identifiers

PMID: 41587019
DOI: 10.1007/s43440-026-00829-7
PMCID: PMC12975861

Genes

USP30

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 1 Sept 2026.

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