Impairment of Lysosome Function and Autophagy in Rare Neurodegenerative Diseases.

Journal of Molecular Biology
Frédéric Darios, Giovanni Stevanin

Abstract

Rare genetic diseases affect a limited number of patients, but their etiology is often known, facilitating the development of reliable animal models and giving the opportunity to investigate physiopathology. Lysosomal storage disorders are a group of rare diseases due to primary alteration of lysosome function. These diseases are often associated with neurological symptoms, which highlighted the importance of lysosome in neurodegeneration. Likewise, other groups of rare neurodegenerative diseases also present lysosomal alteration. Lysosomes fuse with autophagosomes and endosomes to allow the degradation of their content thanks to hydrolytic enzymes. It has emerged that alteration of the autophagy-lysosome pathway could play a critical role in neuronal death in many neurodegenerative diseases. Using a repertoire of selected rare neurodegenerative diseases, we highlight that a variety of alterations of the autophagy-lysosome pathway are associated with neuronal death. Yet, in most cases, it is still unclear why alteration of this pathway can lead to neurodegeneration.

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Citations

Sep 2, 2020·International Journal of Molecular Sciences·Andrés Felipe LealAngela Johana Espejo-Mojica
Feb 14, 2021·Cells·Sara Marmolejo-Martínez-ArteseroDavid Romeo-Guitart
May 5, 2021·Lipids in Health and Disease·Muna Abed RabboJohnny Stiban
May 16, 2021·Journal of Hematology & Oncology·Ziqi ZhangXiawei Wei
May 22, 2021·Neuroscience Bulletin·Linan ZhengWenan Wang
Jun 8, 2021·Frontiers in Cell and Developmental Biology·Ping-Shuang LuShao-Chen Sun
Jul 2, 2021·The Journal of Clinical Investigation·Alpana SinghSubhash Kulkarni
Aug 14, 2021·Ecotoxicology and Environmental Safety·Yue WangShao-Chen Sun

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Methods Mentioned

BETA
dissection
nucleotide exchange
electron microscopy
GTPase
GTPases
nuclear translocation

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