Karyoptosis mediates cell death and neurodegeneration upon proteotoxic stress
M Fanto, R Casterton, Y Hu, A Martinez-Cotrina… - 2023 - researchsquare.com
Lysosomal clearance of proteotoxic stressors through autophagy is an essential process
underlying cellular homeostasis. Its failure can lead to cell death 1, 2, by initiating apoptotic …
underlying cellular homeostasis. Its failure can lead to cell death 1, 2, by initiating apoptotic …
Investigating the spread and toxicity of glycine-alanine dipeptides in C9orf72 ALS/FTD using Drosophila melanogaster
J Morón Oset - 2022 - kups.ub.uni-koeln.de
Hexanucleotide repeat expansions of variable size in C9orf72 are the most prevalent
genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) …
genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) …
Nuclear transport under stress: direct and indirect effects of polyPR and polyGA derail nuclear transport in C9Orf72 yeast models
M Semmelink - 2022 - research.rug.nl
Nucleocytoplasmic transport is the active process of nuclear transport receptors bringing
molecules across the nuclear envelope through nuclear pores. In this work I measured the …
molecules across the nuclear envelope through nuclear pores. In this work I measured the …
Study of DUX4 as a novel biomarker in Amyotrophic Lateral Sclerosis
E Duranti - 2023 - boa.unimib.it
Amyotrophic lateral sclerosis (ALS) is a degenerative neuromuscular disease of unknown
etiology characterized by the impairment of both upper and lower motor neurons. The risk …
etiology characterized by the impairment of both upper and lower motor neurons. The risk …
The role of extracellular vesicles in the progression of ALS and their potential as biomarkers and therapeutic agents with which to combat the disease
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that impairs motor
neuron function, leading to severe muscular atrophy. The non-cell autonomous and …
neuron function, leading to severe muscular atrophy. The non-cell autonomous and …
[PDF][PDF] Dysfunctional nucleocytoplasmic transport dynamics in amyotrophic lateral sclerosis and frontotemporal dementia caused by mutation in C9ORF72
M Salcher-Konrad - 2023 - kclpure.kcl.ac.uk
A mutation in the C9ORF72 gene is the most common genetic cause of the devastating
disorders amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Currently …
disorders amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Currently …
The C9orf72 dipeptide repeat protein poly-glycine-alanine sequesters nucleoporins and disrupts nuclear membranes
K Richter - 2022 - theses.hal.science
In patients with familial amyotrophic lateral sclerosis (ALS) who carry a mutation in the
C9ORF72 gene, dipeptide-repeat proteins accumulate in nervous system tissue. Of these …
C9ORF72 gene, dipeptide-repeat proteins accumulate in nervous system tissue. Of these …
Misfolding at the synapse: A role in amyotrophic lateral sclerosis pathogenesis?
JS Lum, JJ Yerbury - Frontiers in Molecular Neuroscience, 2022 - frontiersin.org
A growing wave of evidence has placed the concept of protein homeostasis at the center of
the pathogenesis of amyotrophic lateral sclerosis (ALS). This is due primarily to the …
the pathogenesis of amyotrophic lateral sclerosis (ALS). This is due primarily to the …
ENGINEERING KAPβ2 TO DISAGGREGATE DISEASE-ASSOCIATED PROTEINS
C Fare - 2022 - repository.upenn.edu
Neurodegenerative diseases result from a failure to maintain proteostasis, and a major
hallmark of neurodegeneration is the accumulation of aggregated protein deposits in the …
hallmark of neurodegeneration is the accumulation of aggregated protein deposits in the …
Importin-α2 mediates brain development, learning and memory consolidation in Drosophila
CN Serway, BS Dunkelberger, D Del Padre… - Journal of …, 2020 - Taylor & Francis
Neuronal development and memory consolidation are conserved processes that rely on
nuclear-cytoplasmic transport of signaling molecules to regulate gene activity and initiate …
nuclear-cytoplasmic transport of signaling molecules to regulate gene activity and initiate …