Autophagy, lysosomes, and ALS/FTD

Şentürk M, Mao D, Bellen HJ. Loss of proteins associated with amyotrophic lateral sclerosis affect lysosomal acidification via different routes. Autophagy. 2019 Apr 28:1-3. PubMed
PMID: 31032688.

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."