[HTML][HTML] In situ structure of neuronal C9orf72 poly-GA aggregates reveals proteasome recruitment
Protein aggregation and dysfunction of the ubiquitin-proteasome system are hallmarks of
many neurodegenerative diseases. Here, we address the elusive link between these …
many neurodegenerative diseases. Here, we address the elusive link between these …
Proximity proteomics of C9orf72 dipeptide repeat proteins identifies molecular chaperones as modifiers of poly-GA aggregation
F Liu, D Morderer, MC Wren… - Acta Neuropathologica …, 2022 - Springer
The most common inherited cause of two genetically and clinico-pathologically overlapping
neurodegenerative diseases, amyotrophic lateral sclerosis (ALS) and frontotemporal …
neurodegenerative diseases, amyotrophic lateral sclerosis (ALS) and frontotemporal …
Cell‐to‐cell transmission of C9orf72 poly‐(Gly‐Ala) triggers key features of ALS/FTD
B Khosravi, KD LaClair, H Riemenschneider… - The EMBO …, 2020 - embopress.org
The C9orf72 repeat expansion causes amyotrophic lateral sclerosis and frontotemporal
dementia, but the poor correlation between C9orf72‐specific pathology and TDP‐43 …
dementia, but the poor correlation between C9orf72‐specific pathology and TDP‐43 …
C9orf72 FTLD/ALS-associated Gly-Ala dipeptide repeat proteins cause neuronal toxicity and Unc119 sequestration
S May, D Hornburg, MH Schludi, T Arzberger… - Acta …, 2014 - Springer
Hexanucleotide repeat expansion in C9orf72 is the most common pathogenic mutation in
patients with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration …
patients with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration …
Proteomics and C9orf72 neuropathology identify ribosomes as poly-GR/PR interactors driving toxicity
H Hartmann, D Hornburg, M Czuppa… - Life science …, 2018 - life-science-alliance.org
Frontotemporal dementia and amyotrophic lateral sclerosis patients with C9orf72 mutation
show cytoplasmic poly-GR and poly-PR aggregates. Short poly-(Gly-Arg) and poly-(Pro …
show cytoplasmic poly-GR and poly-PR aggregates. Short poly-(Gly-Arg) and poly-(Pro …
Antibodies inhibit transmission and aggregation of C9orf72 poly‐GA dipeptide repeat proteins
Cell‐to‐cell transmission of protein aggregates is an emerging theme in neurodegenerative
disease. Here, we analyze the dipeptide repeat (DPR) proteins that form neuronal inclusions …
disease. Here, we analyze the dipeptide repeat (DPR) proteins that form neuronal inclusions …
C9ORF72-ALS/FTD-associated poly (GR) binds Atp5a1 and compromises mitochondrial function in vivo
SY Choi, R Lopez-Gonzalez, G Krishnan… - Nature …, 2019 - nature.com
The GGGGCC repeat expansion in C9ORF72 is the most common genetic cause of
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). However, it is not …
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). However, it is not …
C9orf72 dipeptide repeats impair the assembly, dynamics, and function of membrane-less organelles
Expansion of a hexanucleotide repeat GGGGCC (G 4 C 2) in C9ORF72 is the most common
cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Transcripts …
cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Transcripts …
PolyGR and polyPR knock-in mice reveal a conserved neuroprotective extracellular matrix signature in C9orf72 ALS/FTD neurons
C Milioto, M Carcolé, A Giblin, R Coneys, O Attrebi… - Nature …, 2024 - nature.com
Dipeptide repeat proteins are a major pathogenic feature of C9orf72 amyotrophic lateral
sclerosis (C9ALS)/frontotemporal dementia (FTD) pathology, but their physiological impact …
sclerosis (C9ALS)/frontotemporal dementia (FTD) pathology, but their physiological impact …
C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins
Neuronal inclusions of poly (GA), a protein unconventionally translated from G4C2 repeat
expansions in C9ORF72, are abundant in patients with frontotemporal dementia (FTD) and …
expansions in C9ORF72, are abundant in patients with frontotemporal dementia (FTD) and …