C9orf72 arginine-rich dipeptide repeat proteins disrupt karyopherin-mediated nuclear import
Disruption of nucleocytoplasmic transport is increasingly implicated in the pathogenesis of
neurodegenerative diseases, including ALS caused by a C9orf72 hexanucleotide repeat …
neurodegenerative diseases, including ALS caused by a C9orf72 hexanucleotide repeat …
Nuclear import receptors directly bind to arginine-rich dipeptide repeat proteins and suppress their pathological interactions
S Hutten, S Usluer, B Bourgeois, F Simonetti, HM Odeh… - Cell reports, 2020 - cell.com
Nuclear import receptors, also called importins, mediate nuclear import of proteins and
chaperone aggregation-prone cargoes (eg, neurodegeneration-linked RNA-binding …
chaperone aggregation-prone cargoes (eg, neurodegeneration-linked RNA-binding …
C9orf72-generated poly-GR and poly-PR do not directly interfere with nucleocytoplasmic transport
J Vanneste, T Vercruysse, S Boeynaems, A Sicart… - Scientific reports, 2019 - nature.com
Repeat expansions in the C9orf72 gene cause amyotrophic lateral sclerosis and
frontotemporal dementia characterized by dipeptide-repeat protein (DPR) inclusions. The …
frontotemporal dementia characterized by dipeptide-repeat protein (DPR) inclusions. The …
Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD
S Boeynaems, E Bogaert, E Michiels, I Gijselinck… - Scientific reports, 2016 - nature.com
Hexanucleotide repeat expansions in C9orf72 are the most common cause of amyotrophic
lateral sclerosis (ALS) and frontotemporal degeneration (FTD)(c9ALS/FTD). Unconventional …
lateral sclerosis (ALS) and frontotemporal degeneration (FTD)(c9ALS/FTD). Unconventional …
SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits
Hexanucleotide repeat expansions in the C9ORF72 gene are the commonest known
genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia. Expression of …
genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia. Expression of …
Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS
C9orf72 mutations are the most common cause of amyotrophic lateral sclerosis (ALS) and
frontotemporal dementia (FTD). Dipeptide repeat proteins (DPRs) produced by …
frontotemporal dementia (FTD). Dipeptide repeat proteins (DPRs) produced by …
A cell-penetrant peptide blocking C9ORF72-repeat RNA nuclear export reduces the neurotoxic effects of dipeptide repeat proteins
LM Castelli, YH Lin, A Sanchez-Martinez… - Science Translational …, 2023 - science.org
Hexanucleotide repeat expansions in C9ORF72 are the most common genetic cause of
familial amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Studies …
familial amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Studies …
C9orf72-derived arginine-containing dipeptide repeats associate with axonal transport machinery and impede microtubule-based motility
L Fumagalli, FL Young, S Boeynaems, M De Decker… - Science …, 2021 - science.org
A hexanucleotide repeat expansion in the C9orf72 gene is the most common genetic cause
of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). How this mutation …
of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). How this mutation …
C9orf72-derived arginine-rich poly-dipeptides impede phase modifiers
H Nanaura, H Kawamukai, A Fujiwara… - Nature …, 2021 - nature.com
Nuclear import receptors (NIRs) not only transport RNA-binding proteins (RBPs) but also
modify phase transitions of RBPs by recognizing nuclear localization signals (NLSs). Toxic …
modify phase transitions of RBPs by recognizing nuclear localization signals (NLSs). Toxic …
[HTML][HTML] Stress granule assembly disrupts nucleocytoplasmic transport
Defects in nucleocytoplasmic transport have been identified as a key pathogenic event in
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) mediated by a …
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) mediated by a …