Results 1 to 10 of about 13,747 (162)
Mislocalization of Nucleocytoplasmic Transport Proteins in Human Huntington’s Disease PSC-Derived Striatal Neurons [PDF]
Frontiers in Cellular Neuroscience, 2021 Huntington’s disease (HD) is an inherited neurodegenerative disorder caused by a CAG repeat expansion in the huntingtin gene (HTT). Disease progression is characterized by the loss of vulnerable neuronal populations within the striatum.
Jenny Lange +12 more
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Brain Sciences, 2021 Amyotrophic lateral sclerosis (ALS) is a progressive and fatal neurodegenerative disease with available treatments only marginally slowing progression or improving survival.
Melina Ramic +6 more
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Exercise attenuates age-associated changes in motoneuron number, nucleocytoplasmic transport proteins and neuromuscular health. [PDF]
Geroscience, 2018 Gillon A +4 more
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Cellular Stress Induces Nucleocytoplasmic Transport Deficits Independent of Stress Granules
Biomedicines, 2022 Stress granules are non-membrane bound granules temporarily forming in the cytoplasm in response to stress. Proteins of the nucleocytoplasmic transport machinery were found in these stress granules and it was suggested that stress granules contribute to ...
Joni Vanneste +5 more
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Scientific Reports, 2022 A repeat expansion in C9orf72 is the major cause of both frontotemporal dementia and amyotrophic lateral sclerosis, accounting for approximately 1 in 12 cases of either disease. The expansion is translated to produce five dipeptide repeat proteins (DPRs)
Sarah Ryan +3 more
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Altered nucleocytoplasmic proteome and transcriptome distributions in an in vitro model of amyotrophic lateral sclerosis. [PDF]
PLoS ONE, 2017 Aberrant nucleocytoplasmic localization of proteins has been implicated in many neurodegenerative diseases. Evidence suggests that cytoplasmic mislocalization of nuclear proteins such as transactive response DNA-binding protein 43 (TDP-43) and fused in ...
Jee-Eun Kim +9 more
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Frontiers in Microbiology, 2022 The nuclear localization signal (NLS) and nuclear export signal (NES) are key signatures of proteins for controlling nuclear import and export. The NIb protein of turnip mosaic virus (TuMV) is an RNA-dependent RNA polymerase (RdRP) that is absolutely ...
Mingzhen Zhang +11 more
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Understanding the role of mechanics in nucleocytoplasmic transport
APL Bioengineering, 2022 Cell nuclei are submitted to mechanical forces, which in turn affect nuclear and cell functions. Recent evidence shows that a crucial mechanically regulated nuclear function is nucleocytoplasmic transport, mediated by nuclear pore complexes (NPCs ...
Ion Andreu +3 more
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C9orf72 Toxic Species Affect ArfGAP-1 Function
Cells, 2023 Compelling evidence indicates that defects in nucleocytoplasmic transport contribute to the pathogenesis of amyotrophic lateral sclerosis (ALS). In particular, hexanucleotide (G4C2) repeat expansions in C9orf72, the most common cause of genetic ALS, have
Simona Rossi +9 more
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Regulating Phase Transition in Neurodegenerative Diseases by Nuclear Import Receptors
Biology, 2022 RNA-binding proteins (RBPs) with a low-complexity prion-like domain (PLD) can undergo aberrant phase transitions and have been implicated in neurodegenerative diseases such as ALS and FTD.
Amandeep Girdhar, Lin Guo
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