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Review
. 2024 Feb;40(2):167-175.
doi: 10.1051/medsci/2023220. Epub 2024 Feb 27.

[Optineurin and mitochondrial dysfunction in neurodegeneration]

[Article in French]
Baptiste D'Urso  1 , Robert Weil  2 , Pierre Génin  2
Affiliations
Free article
Review

[Optineurin and mitochondrial dysfunction in neurodegeneration]

[Article in French]
Baptiste D'Urso et al. Med Sci (Paris). 2024 Feb.
Free article

Abstract
in English, French

Optineurin (OPTN) is a multifunctional protein playing a crucial role as a receptor in selective autophagy. OPTN gene mutations are linked to diseases such as normal-tension glaucoma and amyotrophic lateral sclerosis. Recognized as a critical receptor for mitophagy, OPTN is pivotal in selectively degrading damaged mitochondria. This process is essential to prevent their accumulation, the generation of reactive oxygen species, and the release of pro-apoptotic factors. Mitophagy's quality control is governed by the PINK1 kinase and the cytosolic ubiquitin ligase Parkin, whose mutations are associated with Parkinson's disease. This review highlights recent insights emphasizing OPTN's role in mitophagy and its potential involvement in neurodegenerative diseases.

Title: L’optineurine et les dysfonctionnements mitochondriaux dans la neurodégénérescence.

Abstract: L’optineurine (OPTN) est une protéine multifonctionnelle jouant un rôle crucial en tant que récepteur dans l’autophagie sélective. Les mutations du gène OPTN sont liées à des maladies telles que le glaucome à tension normale et la sclérose latérale amyotrophique. L’OPTN exerce une fonction essentielle dans la dégradation sélective des mitochondries endommagées. Ce processus est requis pour empêcher leur accumulation, la production d’espèces réactives de l’oxygène et la libération de facteurs pro-apoptotiques. Le contrôle de la qualité de la mitophagie est orchestré par la kinase PINK1 et la ligase de l’ubiquitine cytosolique Parkin, dont les mutations sont associées à la maladie de Parkinson. Cette revue met en lumière des perspectives récentes soulignant le rôle de l’OPTN dans la mitophagie et son implication potentielle dans les maladies neurodégénératives.

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References

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