Şentürk, M., Mao, D., Bellen, H.J. (2019). Loss of proteins associated with amyotrophic lateral sclerosis affects lysosomal acidification via different routes.  Autophagy 15(8): 1467--1469.

FBrf0242755

Note

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.

PMC6613899 (PMC) (EuropePMC)