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Article

Pathways controlling neurotoxicity and proteostasis in mitochondrial complex I deficiency.

Human molecular genetics - 4 May 2024

Nithianandam Vanitha, Sarkar Souvarish, Feany Mel B

Abstract excerpt

Neuromuscular disorders caused by dysfunction of the mitochondrial respiratory chain are common, severe and untreatable. We recovered a number of mitochondrial genes, including electron transport chain components, in a large forward genetic screen for mutations causing age-related neurodegeneration in the context of proteostasis dysfunction. We created a model of complex I deficiency in the Drosophila retina to...

Topics

Animals
Proteostasis
Electron Transport Complex I
Mitochondria
Drosophila Proteins
Mitochondrial Diseases
Disease Models, Animal
Humans
Proteasome Endopeptidase Complex
Autophagy
Oxidative Stress

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Drosophila melanogaster

Mutation

Lysosomes

Drosophila

Signal Transduction

Source

Open in PubMed Open full text

Identifiers

PMID: 38324746
DOI: 10.1093/hmg/ddae018
PMCID: PMC11070137

Genes

RIOK1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 31 Aug 2026.

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