Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

March 18, 2020
Review
Open Access

Traffic jam at the nuclear pore: All roads lead to nucleocytoplasmic transport defects in ALS/FTD

Neurobiology of Disease
Claudia FalliniWilfried Rossoll
October 22, 2016
Open Access

C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles

Cell
Kyung-Ha LeeJ Paul Taylor
May 26, 2016

Mechanisms of membrane-pore formation by amyloidogenic peptides in ALS

Chemistry : a European Journal
Charles H ChenMartin B Ulmschneider
October 22, 2016
Open Access

Toxic PR Poly-Dipeptides Encoded by the C9orf72 Repeat Expansion Target LC Domain Polymers

Cell
Yi LinSteven L McKnight
September 30, 2019
Preprint
Open Access

C9orf72 arginine-rich dipeptide repeat proteins disrupt importin β-mediated nuclear import

BioRxiv : the Preprint Server for Biology
Lindsey R HayesJeffrey D Rothstein
December 29, 2020
Open Access

LSM12-EPAC1 defines a neuroprotective pathway that sustains the nucleocytoplasmic RAN gradient

PLoS Biology
Jongbo LeeChunghun Lim
February 6, 2017
Comment / Editorial
Open Access

A PR plug for the nuclear pore in amyotrophic lateral sclerosis

Proceedings of the National Academy of Sciences of the United States of America
J Paul Taylor
September 4, 2020
Open Access

C9orf72 poly(GR) aggregation induces TDP-43 proteinopathy

Science Translational Medicine
Casey N CookLeonard Petrucelli
August 24, 2020

The pathophysiology of neurodegenerative disease: Disturbing the balance between phase separation and irreversible aggregation

Progress in Molecular Biology and Translational Science
Chelsea J WebberBenjamin Wolozin
January 4, 2017
Open Access

Cytoplasmic poly-GA aggregates impair nuclear import of TDP-43 in C9orf72 ALS/FTLD

Human Molecular Genetics
Bahram KhosraviDieter Edbauer
November 6, 2020
Open Access

Sigma-1 receptor chaperones rescue nucleocytoplasmic transport deficit seen in cellular and Drosophila ALS/FTD models

Nature Communications
Pin-Tse LeeTsung-Ping Su
February 25, 2019
Preprint
Open Access

Pleiotropic requirements for human TDP-43 in the regulation of cell and organelle homeostasis

BioRxiv : the Preprint Server for Biology
Shawn M Ferguson, Agnes Roczniak-Ferguson
February 12, 2019
Review
Open Access

The coming-of-age of nucleocytoplasmic transport in motor neuron disease and neurodegeneration

Cellular and Molecular Life Sciences : CMLS
Paulo A Ferreira
January 11, 2017
Open Access

Toxic PRn poly-dipeptides encoded by the C9orf72 repeat expansion block nuclear import and export

Proceedings of the National Academy of Sciences of the United States of America
Kevin Y ShiSteven L McKnight
March 28, 2017

Aberrant distributions of nuclear pore complex proteins in ALS mice and ALS patients

Neuroscience
Jingwei ShangKoji Abe
July 17, 2020

G4C2 Repeat RNA Initiates a POM121-Mediated Reduction in Specific Nucleoporins in C9orf72 ALS/FTD.

Neuron
Alyssa N CoyneJeffrey D Rothstein
March 21, 2018
Review
Open Access

DNA plasticity and damage in amyotrophic lateral sclerosis

Neural Regeneration Research
Diane PenndorfAlexandra Kretz
December 29, 2016

Dynamic mislocalizations of nuclear pore complex proteins after focal cerebral ischemia in rat

Journal of Neuroscience Research
Qian LiKoji Abe

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