Loss of proteins associated with amyotrophic lateral sclerosis affect lysosomal acidification via different routes.

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

Source: Autophagy - April 27, 2019 Category: Cytology Authors: Şentürk M, Mao D, Bellen HJ Tags: Autophagy Source Type: research