Abstract
Abnormal accumulation of proteins is a hallmark of a variety of neurological diseases including amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Maintenance of protein homeostasis (proteostasis) in neurons via proteasomal and macroautophagy/autophagy-lysosomal degradation is thought to be central for proper neuronal function and survival. We recently reported evolutionarily conserved roles for two ALS-linked proteins, UBQLN2 (ubiquilin 2) and VAPB, in regulation of lysosomal degradation. Ubiquilins are required for v-ATPase-mediated lysosomal acidification, whereas VAPs are required for the PtdIns4P-mediated endo-lysosomal trafficking pathway.
Original language | English (US) |
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Pages (from-to) | 1467-1469 |
Number of pages | 3 |
Journal | Autophagy |
Volume | 15 |
Issue number | 8 |
DOIs |
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State | Published - Aug 3 2019 |
Externally published | Yes |
Keywords
- ALS
- Drosophila
- ER stress
- lysosomal acidification
- MTOR
- v-ATPase
ASJC Scopus subject areas
- Molecular Biology
- Cell Biology