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Article

Optineurin is an autophagy receptor for damaged mitochondria in parkin-mediated mitophagy that is disrupted by an ALS-linked mutation.

Proceedings of the National Academy of Sciences of the United States of America - 21 Oct 2014

Wong Yvette C, Holzbaur Erika L F

Abstract excerpt

Mitophagy is a cellular quality control pathway in which the E3 ubiquitin ligase parkin targets damaged mitochondria for degradation by autophagosomes. We examined the role of optineurin in mitophagy, as mutations in optineurin are causative for amyotrophic lateral sclerosis (ALS) and glaucoma, diseases in which mitochondrial dysfunction has been implicated. Using live cell imaging, we demonstrate the...

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Identifiers

PMID: 25294927
DOI: 10.1073/pnas.1405752111
PMCID: PMC4210283

Genes

SQSTM1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Optineurin is an autophagy receptor for damaged mitochondria in parkin-mediated mitophagy that is disrupted by an ALS-linked mutation.PMID 25294927Read ↗

Temporal dynamics of PARK2/parkin and OPTN/optineurin recruitment during the mitophagy of damaged mitochondriaSemantic proximity Read ↗

Role of Optineurin in the Mitochondrial Dysfunction: Potential Implications in Neurodegenerative Diseases and CancerSemantic proximity Read ↗

ALS-Related Mutant SOD1 Aggregates Interfere with Mitophagy by Sequestering the Autophagy Receptor Optineurin.Semantic proximity Read ↗

Dynamic recruitment and activation of ALS-associated TBK1 with its target optineurin are required for efficient mitophagy.Semantic proximity Read ↗

Optineurin: A Coordinator of Membrane-Associated Cargo Trafficking and Autophagy.Semantic proximity Read ↗

Glaucoma-Associated Mutations in the Optineurin Gene Have Limited Impact on Parkin-Dependent MitophagySemantic proximity Read ↗

Fundamental roles of the Optineurin gene in the molecular pathology of Amyotrophic Lateral SclerosisSemantic proximity Read ↗

Defects in optineurin- and myosin VI-mediated cellular trafficking in amyotrophic lateral sclerosisSemantic proximity Read ↗

Damaged mitochondria recruit the effector NEMO to activate NF-κB signalingSemantic proximity Read ↗

Emerging views of OPTN (optineurin) function in the autophagic process associated with diseaseSemantic proximity Read ↗

Degradation of engulfed mitochondria is rate-limiting in Optineurin-mediated mitophagy in neurons.Semantic proximity Read ↗

Unconventional initiation of PINK1/Parkin mitophagy by OptineurinSemantic proximity Read ↗

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