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Updated: Jul 2, 2025

Author Spotlight: Establishing a New Fluorescence-Based Protocol for In Vivo Mitochondrial Morphology Analysis in Parkinson's Disease
Published on: June 23, 2023
[Optineurin and mitochondrial dysfunction in neurodegeneration]
Baptiste D'Urso1, Robert Weil2, Pierre Génin2
1CIMI-Paris, UPMC UMRS CR7 - Inserm U1135 - CNRS EMR8255, Faculté de médecine Sorbonne Université site Pitié-Salpêtrière, Paris, France - Sorbonne Université, Faculté des sciences et ingénierie, Paris, France.
Abstract:
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.
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