Synaptic dysfunction in ALS and FTD: anatomical and molecular changes provide insights into mechanisms of disease

PA Gelon, PA Dutchak, CF Sephton - Frontiers in Molecular …, 2022 - frontiersin.org
Synaptic loss is a pathological feature of all neurodegenerative diseases including
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). ALS is a disease of …

[HTML][HTML] New insights into the role of autophagy in retinal and eye diseases

B Villarejo-Zori, JI Jiménez-Loygorri… - Molecular Aspects of …, 2021 - Elsevier
Autophagy is a fundamental homeostatic pathway that mediates the degradation and
recycling of intracellular components. It serves as a key quality control mechanism …

An abundance of free regulatory (19S) proteasome particles regulates neuronal synapses

C Sun, K Desch, B Nassim-Assir, SL Giandomenico… - Science, 2023 - science.org
The proteasome, the major protein-degradation machine in cells, regulates neuronal
synapses and long-term information storage. Here, using super-resolution microscopy, we …

Physiological functions of intracellular protein degradation

E McShane, M Selbach - Annual Review of Cell and …, 2022 - annualreviews.org
While cellular proteins were initially thought to be stable, research over the last decades has
firmly established that intracellular protein degradation is an active and highly regulated …

Logistics of neuronal protein turnover: Numbers and mechanisms

C Sun, EM Schuman - Molecular and Cellular Neuroscience, 2022 - Elsevier
Research in the past twenty years or so has revealed that neurons synthesize and degrade
proteins at their synapses to enable synaptic proteome remodelling on demand and in real …

Presynaptic Protein Synthesis in Brain Function and Disease

PE Castillo, H Jung, E Klann, A Riccio - Journal of Neuroscience, 2023 - Soc Neuroscience
Local protein synthesis in mature brain axons regulates the structure and function of
presynaptic boutons by adjusting the presynaptic proteome to local demands. This crucial …

The disturbance of protein synthesis/degradation homeostasis is a common trait of age-related neurodegenerative disorders

F Di Domenico, C Lanzillotta - … in Protein Chemistry and Structural Biology, 2022 - Elsevier
Protein homeostasis or “proteostasis” represent the process that regulates the balance of the
intracellular functional and “healthy” proteins. Proteostasis is fundamental to preserve …

Systematic analysis of YFP traps reveals common mRNA/protein discordance in neural tissues

JS Titlow, M Kiourlappou, A Palanca, JY Lee… - Journal of Cell …, 2023 - rupress.org
While post-transcriptional control is thought to be required at the periphery of neurons and
glia, its extent is unclear. Here, we investigate systematically the spatial distribution and …

Identification of molecular signatures defines the differential proteostasis response in induced spinal and cranial motor neurons

APZP Fiore, S Maity, L Jeffery, D An, J Rendleman… - Cell reports, 2024 - cell.com
Amyotrophic lateral sclerosis damages proteostasis, affecting spinal and upper motor
neurons earlier than a subset of cranial motor neurons. To aid disease understanding, we …

Synapse integrity and function: Dependence on protein synthesis and identification of potential failure points

LD Cohen, T Ziv, NE Ziv - Frontiers in Molecular Neuroscience, 2022 - frontiersin.org
Synaptic integrity and function depend on myriad proteins-labile molecules with finite
lifetimes that need to be continually replaced with freshly synthesized copies. Here we …