Misrouting of v-ATPase subunit V0a1 dysregulates lysosomal acidification in a neurodegenerative lysosomal storage disease model
oleh: Maria B. Bagh, Shiyong Peng, Goutam Chandra, Zhongjian Zhang, Satya P. Singh, Nagarajan Pattabiraman, Aiyi Liu, Anil B. Mukherjee
| Format: | Article |
|---|---|
| Diterbitkan: | Nature Portfolio 2017-03-01 |
Deskripsi
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.