Nuclear pore complex and nucleocytoplasmic transport disruption in neurodegeneration
Nuclear pore complexes (NPCs) play a critical role in maintaining the equilibrium between
the nucleus and cytoplasm, enabling bidirectional transport across the nuclear envelope …
the nucleus and cytoplasm, enabling bidirectional transport across the nuclear envelope …
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 …
Unraveling the impact of disrupted nucleocytoplasmic transport systems in C9orf72-associated ALS
P McGoldrick, J Robertson - Frontiers in Cellular Neuroscience, 2023 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two adult-onset
neurodegenerative diseases that are part of a common disease spectrum due to clinical …
neurodegenerative diseases that are part of a common disease spectrum due to clinical …
Repeat length of C9orf72-associated glycine–alanine polypeptides affects their toxicity
J Morón-Oset, LKS Fischer, N Jauré, P Zhang… - Acta Neuropathologica …, 2023 - Springer
G4C2 hexanucleotide repeat expansions in a non-coding region of the C9orf72 gene are
the most common cause of familial amyotrophic lateral sclerosis (ALS) and frontotemporal …
the most common cause of familial amyotrophic lateral sclerosis (ALS) and frontotemporal …
Advances in the structure of GGGGCC repeat RNA sequence and its interaction with small molecules and protein partners
X Liu, X Zhao, J He, S Wang, X Shen, Q Liu, S Wang - Molecules, 2023 - mdpi.com
The aberrant expansion of GGGGCC hexanucleotide repeats within the first intron of the
C9orf72 gene represent the predominant genetic etiology underlying amyotrophic lateral …
C9orf72 gene represent the predominant genetic etiology underlying amyotrophic lateral …
Activation of the Keap1/Nrf2 pathway suppresses mitochondrial dysfunction in C9orf72 ALS/FTD in vivo models and patient iNeurons
WH Au, L Miller-Fleming, A Sanchez-Martinez, J Lee… - bioRxiv, 2023 - biorxiv.org
Mitochondrial dysfunction such as excess production of reactive oxygen species (ROS) and
defective mitochondrial dynamics are common features of C9orf72 Amyotrophic Lateral …
defective mitochondrial dynamics are common features of C9orf72 Amyotrophic Lateral …
A nerve-wracking buzz: lessons from Drosophila models of peripheral neuropathy and axon degeneration
MRC Bhattacharya - Frontiers in Aging Neuroscience, 2023 - frontiersin.org
The degeneration of axons and their terminals occurs following traumatic, toxic, or
genetically-induced insults. Common molecular mechanisms unite these disparate triggers …
genetically-induced insults. Common molecular mechanisms unite these disparate triggers …
RNA Dysmetabolism and Repeat-Associated Non-AUG Translation in Frontotemporal Lobar Degeneration/Amyotrophic Lateral Sclerosis due to C9orf72 …
Neuropathological features of frontotemporal dementia and amyotrophic lateral sclerosis
(ALS) due to C9orf72 GGGGCC hexanucleotide repeat expansion include early dipeptide …
(ALS) due to C9orf72 GGGGCC hexanucleotide repeat expansion include early dipeptide …
Phenylalanine-tRNA aminoacylation is compromised by ALS/FTD-associated C9orf72 C4G2 repeat RNA
M Malnar Črnigoj, U Čerček, X Yin, MT Ho… - Nature …, 2023 - nature.com
The expanded hexanucleotide GGGGCC repeat mutation in the C9orf72 gene is the main
genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia. Under one …
genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia. Under one …
Increased Karyopherin Alpha Levels Attenuate Mutant Ataxin-1-Induced Neurodegeneration
EK Ruff, DL Timperman, AA Amador, I Aguirre-Lamus… - bioRxiv, 2023 - biorxiv.org
Neurodegenerative diseases are characterized by the abnormal accumulation of disease-
driving proteins. Emerging evidence suggests that nucleocytoplasmic transport (NCT) …
driving proteins. Emerging evidence suggests that nucleocytoplasmic transport (NCT) …