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

Nat Commun. 2016 Jun 9:7:11803. doi: 10.1038/ncomms11803.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

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

Substances

  • ATP13A2 protein, human
  • Basic Helix-Loop-Helix Leucine Zipper Transcription Factors
  • RNA, Messenger
  • SYT11 protein, human
  • TFEB protein, human
  • Synaptotagmins
  • Mechanistic Target of Rapamycin Complex 1
  • Proton-Translocating ATPases