[HTML][HTML] The role of TDP-43 mislocalization in amyotrophic lateral sclerosis
TR Suk, MWC Rousseaux - Molecular neurodegeneration, 2020 - Springer
Since its discovery as a primary component in cytoplasmic aggregates in post-mortem tissue
of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP …
of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP …
Stress granule subtypes: an emerging link to neurodegeneration
Abstract Stress Granules (SGs) are membraneless cytoplasmic RNA granules, which
contain translationally stalled mRNAs, associated translation initiation factors and multiple …
contain translationally stalled mRNAs, associated translation initiation factors and multiple …
[HTML][HTML] 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 …
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 …
[HTML][HTML] Traffic jam at the nuclear pore: All roads lead to nucleocytoplasmic transport defects in ALS/FTD
Amyotrophic lateral sclerosis (ALS) is a fatal late-onset neurodegenerative disease that
specifically affects the function and survival of spinal and cortical motor neurons. ALS shares …
specifically affects the function and survival of spinal and cortical motor neurons. ALS shares …
Nucleocytoplasmic transport defects in neurodegeneration—Cause or consequence?
S Hutten, D Dormann - Seminars in cell & developmental biology, 2020 - Elsevier
Defects in nucleocytoplasmic transport have been associated with several
neurodegenerative disorders and, in particular, the formation of pathological protein …
neurodegenerative disorders and, in particular, the formation of pathological protein …
[HTML][HTML] Chimeric peptide species contribute to divergent dipeptide repeat pathology in c9ALS/FTD and SCA36
ZT McEachin, TF Gendron, N Raj, M García-Murias… - Neuron, 2020 - cell.com
GGGGCC hexanucleotide repeat expansions (HREs) in C9orf72 cause amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD) and lead to the production of …
sclerosis (ALS) and frontotemporal dementia (FTD) and lead to the production of …
The role of DNA damage response in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a rapidly disabling and fatal neurodegenerative
disease. Due to insufficient disease-modifying treatments, there is an unmet and urgent …
disease. Due to insufficient disease-modifying treatments, there is an unmet and urgent …
[HTML][HTML] Nucleo–cytoplasmic transport defects and protein aggregates in neurodegeneration
G Bitetto, A Di Fonzo - Translational neurodegeneration, 2020 - Springer
In the ongoing process of uncovering molecular abnormalities in neurodegenerative
diseases characterized by toxic protein aggregates, nucleo-cytoplasmic transport defects …
diseases characterized by toxic protein aggregates, nucleo-cytoplasmic transport defects …
[HTML][HTML] Implications of selective autophagy dysfunction for ALS pathology
Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disorder that progressively
affects motor neurons in the brain and spinal cord. Due to the biological complexity of the …
affects motor neurons in the brain and spinal cord. Due to the biological complexity of the …