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The Parkinson's disease-associated genes ATP13A2 and SYT11 regulate autophagy via a common pathway.

Published version
Peer-reviewed

Repository DOI


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Authors

Bento, Carla F 
Ashkenazi, Avraham 
Jimenez-Sanchez, Maria 
Rubinsztein, David C 

Abstract

Forms of Parkinson's disease (PD) are associated with lysosomal and autophagic dysfunction. ATP13A2, which is mutated in some types of early-onset Parkinsonism, has been suggested as a regulator of the autophagy-lysosome pathway. However, little is known about the ATP13A2 effectors and how they regulate this pathway. Here we show that ATP13A2 depletion negatively regulates another PD-associated gene (SYT11) at both transcriptional and post-translational levels. Decreased SYT11 transcription is controlled by a mechanism dependent on MYCBP2-induced ubiquitination of TSC2, which leads to mTORC1 activation and decreased TFEB-mediated transcription of SYT11, while increased protein turnover is regulated by SYT11 ubiquitination and degradation. Both mechanisms account for a decrease in the levels of SYT11, which, in turn, induces lysosomal dysfunction and impaired degradation of autophagosomes. Thus, we propose that ATP13A2 and SYT11 form a new functional network in the regulation of the autophagy-lysosome pathway, which is likely to contribute to forms of PD-associated neurodegeneration.

Description

Keywords

Autophagy, Basic Helix-Loop-Helix Leucine Zipper Transcription Factors, HeLa Cells, Humans, Lysosomes, Mechanistic Target of Rapamycin Complex 1, Parkinson Disease, Proton-Translocating ATPases, RNA, Messenger, Signal Transduction, Synaptotagmins, Ubiquitination

Journal Title

Nat Commun

Conference Name

Journal ISSN

2041-1723
2041-1723

Volume Title

7

Publisher

Springer Science and Business Media LLC
Sponsorship
Wellcome Trust (095317/Z/11/Z)
Addenbrooke's Charitable Trust (ACT) (PF15/DR/9342)
Federation of the European Biochemical Societies (FEBS) (unknown)
Medical Research Council (MC_G1000734)
Wellcome Trust (100140/Z/12/Z)
We are grateful to Wellcome Trust (Principal Research Fellowship to DCR (095317/Z/11/Z), Wellcome Trust/MRC Strategic Award in Neurodegeneration, and Wellcome Trust Strategic Grant to Cambridge Institute for Medical Research (100140/Z/12/Z)), Addenbrooke’s Charitable Trust, and FEBS (Long-Term Fellowship to AA) for funding.