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 …
Triad of TDP43 control in neurodegeneration: autoregulation, localization and aggregation
P Tziortzouda, L Van Den Bosch, F Hirth - Nature Reviews …, 2021 - nature.com
Cytoplasmic aggregation of TAR DNA-binding protein 43 (TDP43; also known as TARDBP
or TDP-43) is a key pathological feature of several neurodegenerative diseases, including …
or TDP-43) is a key pathological feature of several neurodegenerative diseases, including …
Monomerization of TDP-43 is a key determinant for inducing TDP-43 pathology in amyotrophic lateral sclerosis
K Oiwa, S Watanabe, K Onodera, Y Iguchi… - Science …, 2023 - science.org
The cytoplasmic aggregation of TAR DNA binding protein-43 (TDP-43), also known as TDP-
43 pathology, is the pathological hallmark of amyotrophic lateral sclerosis (ALS). However …
43 pathology, is the pathological hallmark of amyotrophic lateral sclerosis (ALS). However …
Mitochondrial bioenergetic deficits in C9orf72 amyotrophic lateral sclerosis motor neurons cause dysfunctional axonal homeostasis
Axonal dysfunction is a common phenotype in neurodegenerative disorders, including in
amyotrophic lateral sclerosis (ALS), where the key pathological cell-type, the motor neuron …
amyotrophic lateral sclerosis (ALS), where the key pathological cell-type, the motor neuron …
[HTML][HTML] NUP62 localizes to ALS/FTLD pathological assemblies and contributes to TDP-43 insolubility
AM Gleixner, BM Verdone, CG Otte… - Nature …, 2022 - nature.com
Abstract A G4C2 hexanucleotide repeat expansion in the C9orf72 gene is the most common
genetic cause of ALS and FTLD (C9-ALS/FTLD) with cytoplasmic TDP-43 inclusions …
genetic cause of ALS and FTLD (C9-ALS/FTLD) with cytoplasmic TDP-43 inclusions …
[PDF][PDF] 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 …
Cellular and physiological functions of C9ORF72 and implications for ALS/FTD
W Pang, F Hu - Journal of neurochemistry, 2021 - Wiley Online Library
The hexanucleotide repeat expansion (HRE) in the C9ORF72 gene is the main cause of two
tightly linked neurodegenerative diseases, amyotrophic lateral sclerosis (ALS) and …
tightly linked neurodegenerative diseases, amyotrophic lateral sclerosis (ALS) and …
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 …
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] 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 …