Plekhg5-regulated autophagy of synaptic vesicles reveals a pathogenic mechanism in motoneuron disease

P Lüningschrör, B Binotti, B Dombert… - Nature …, 2017 - nature.com
Autophagy-mediated degradation of synaptic components maintains synaptic homeostasis
but also constitutes a mechanism of neurodegeneration. It is unclear how autophagy of …

Endophilin-A/SH3GL2 calcium switch for synaptic autophagy induction is impaired by a Parkinson's risk variant

M Decet, SF Soukup - Autophagy, 2024 - Taylor & Francis
At the synapse, proteins are reused several times during neuronal activity, causing a decline
in protein function over time. Although emerging evidence supports a role of autophagy in …

Inhibition of lysosome membrane recycling causes accumulation of gangliosides that contribute to neurodegeneration

M Boutry, J Branchu, C Lustremant, C Pujol, J Pernelle… - Cell reports, 2018 - cell.com
Lysosome membrane recycling occurs at the end of the autophagic pathway and requires
proteins that are mostly encoded by genes mutated in neurodegenerative diseases …

Inhibition of retrograde transport modulates misfolded protein accumulation and clearance in motoneuron diseases

R Cristofani, V Crippa, P Rusmini, ME Cicardi… - Autophagy, 2017 - Taylor & Francis
Motoneuron diseases, like spinal bulbar muscular atrophy (SBMA) and amyotrophic lateral
sclerosis (ALS), are associated with proteins that because of gene mutation or peculiar …

HspB8 mutation causing hereditary distal motor neuropathy impairs lysosomal delivery of autophagosomes

AS Kwok, K Phadwal, BJ Turner… - Journal of …, 2011 - Wiley Online Library
J. Neurochem.(2011) 10.1111/j. 1471‐4159.2011. 07521. x Abstract HspB8, a small heat‐
shock protein implicated in autophagy, is mutated in patients with distal hereditary motor …

XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy

C Hetz, P Thielen, S Matus, M Nassif… - Genes & …, 2009 - genesdev.cshlp.org
Mutations in superoxide dismutase-1 (SOD1) cause familial amyotrophic lateral sclerosis
(fALS). Recent evidence implicates adaptive responses to endoplasmic reticulum (ER) …

BAG3 and SYNPO (synaptopodin) facilitate phospho-MAPT/Tau degradation via autophagy in neuronal processes

C Ji, M Tang, C Zeidler, J Höhfeld, GVW Johnson - Autophagy, 2019 - Taylor & Francis
ABSTRACT A major cellular catabolic pathway in neurons is macroautophagy/autophagy,
through which misfolded or aggregation-prone proteins are sequestered into …

The GTPase Rab26 links synaptic vesicles to the autophagy pathway

B Binotti, NJ Pavlos, D Riedel, D Wenzel… - elife, 2015 - elifesciences.org
Small GTPases of the Rab family not only regulate target recognition in membrane traffic but
also control other cellular functions such as cytoskeletal transport and autophagy. Here we …

Impaired autophagy in motor neurons: a final common mechanism of injury and death

MA Gonzalez Porras, GC Sieck, CB Mantilla - Physiology, 2018 - journals.physiology.org
Autophagy is a cellular digestion process that contributes to cellular homeostasis and
adaptation by the elimination of proteins and damaged organelles. Evidence suggests that …

Mice deficient in Epg5 exhibit selective neuronal vulnerability to degeneration

H Zhao, YG Zhao, X Wang, L Xu, L Miao, D Feng… - Journal of Cell …, 2013 - rupress.org
The molecular mechanism underlying the selective vulnerability of certain neuronal
populations associated with neurodegenerative diseases remains poorly understood. Basal …