Nature Communications (Mar 2017)

Misrouting of v-ATPase subunit V0a1 dysregulates lysosomal acidification in a neurodegenerative lysosomal storage disease model

  • Maria B. Bagh,
  • Shiyong Peng,
  • Goutam Chandra,
  • Zhongjian Zhang,
  • Satya P. Singh,
  • Nagarajan Pattabiraman,
  • Aiyi Liu,
  • Anil B. Mukherjee

DOI
https://doi.org/10.1038/ncomms14612
Journal volume & issue
Vol. 8, no. 1
pp. 1 – 16

Abstract

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Lysosomal acidification defects have been implicated in various neurodegenerative disorders. Baghet al. show that the V0a1 subunit of v-ATPase requires palmitoylation for correct sorting and trafficking to the lysosome membrane, and that such a process is impaired in a mouse model of a devastating neurodegenerative lysosomal storage disease, INCL.