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.

October 28, 2019

Oncogenic potential of nucleoporins in non-hematological cancers: recent update beyond chromosome translocation and gene fusion

Journal of Cancer Research and Clinical Oncology
Adhiraj Roy, Gopeshwar Narayan
March 18, 2020

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

Kyung-Ha LeeJ Paul Taylor
October 22, 2016
Open Access

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

Yi LinSteven L McKnight
October 24, 2018
Open Access

Coding mutations in NUS1 contribute to Parkinson's disease

Proceedings of the National Academy of Sciences of the United States of America
Ji-Feng GuoBei-Sha Tang
September 30, 2019
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 14, 2018
Case Report

Biallelic mutations in nucleoporin NUP88 cause lethal fetal akinesia deformation sequence

PLoS Genetics
Edith BonninBirthe Fahrenkrog
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
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
February 25, 2019
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

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

Jingwei ShangKoji Abe
July 17, 2020

G4 C2 Repeat RNA Initiates a POM121-Mediated Reduction in Specific Nucleoporins in C9orf72 ALS/FTD

Alyssa N CoyneJeffrey D Rothstein
March 21, 2018

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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