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.

September 27, 2021

Nuclei Isolation and Super-Resolution Structured Illumination Microscopy for Examining Nucleoporin Alterations in Human Neurodegeneration.

Journal of Visualized Experiments : JoVE
Alyssa N Coyne, Jeffrey D Rothstein
September 10, 2021
Open Access

Measuring and Interpreting Nuclear Transport in Neurodegenerative Disease-The Example of C9orf72 ALS.

International Journal of Molecular Sciences
Marije F W SemmelinkLiesbeth M Veenhoff
September 1, 2021

Nuclear-Import Receptors Counter Deleterious Phase Transitions in Neurodegenerative Disease.

Journal of Molecular Biology
Hana M OdehJames Shorter
August 25, 2021
Open Access

Loss of nucleoporin Nup50 is a risk factor for amyotrophic lateral sclerosis

MedRxiv : the Preprint Server for Health Sciences
S. MegatLuc Dupuis
August 24, 2021
Open Access

Nuclear RNA binding regulates TDP-43 nuclear localization and passive nuclear export

BioRxiv : the Preprint Server for Biology
Lauren DuanLindsey R. Hayes
July 30, 2021

Nuclear accumulation of CHMP7 initiates nuclear pore complex injury and subsequent TDP-43 dysfunction in sporadic and familial ALS.

Science Translational Medicine
Alyssa N CoyneJeffrey D Rothstein
June 25, 2021

Synthetic hydrogel mimics of the nuclear pore complex for the study of nucleocytoplasmic transport defects in C9orf72 ALS/FTD.

Analytical and Bioanalytical Chemistry
Alicia K FriedmanLane A Baker
June 1, 2021
Open Access

Traumatic injury compromises nucleocytoplasmic transport and leads to TDP-43 pathology.

Eric N AndersonUdai Pandey
May 24, 2021
Open Access

Mutation Screening of the GLE1 Gene in a Large Chinese Cohort of Amyotrophic Lateral Sclerosis Patients.

Frontiers in Neuroscience
Yanran LiXusheng Huang
May 10, 2021
Open Access

Altered Phase Separation and Cellular Impact in C9orf72-Linked ALS/FTD.

Frontiers in Cellular Neuroscience
Daniel A SolomonSarah Mizielinska
May 1, 2021
Open Access

Nucleocytoplasmic Transport: Regulatory Mechanisms and the Implications in Neurodegeneration.

International Journal of Molecular Sciences
Baojin Ding, Masood Sepehrimanesh
March 21, 2021
Open Access

Nuclear lamina invaginations are not a pathological feature of C9orf72 ALS/FTD.

Acta Neuropathologica Communications
Alyssa N Coyne, Jeffrey D Rothstein
February 20, 2021
Open Access

Nup62 is recruited to pathological condensates and promotes TDP-43 insolubility in C9orf72 and sporadic ALS/FTLD.

Amanda GleixnerChristopher Donnelly
December 29, 2020
Open Access

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

PLoS Biology
Jongbo LeeChunghun Lim
December 11, 2020
Open Access

TFEB/Mitf links impaired nuclear import to autophagolysosomal dysfunction in C9-ALS.

Kathleen M CunninghamThomas E Lloyd
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
October 9, 2020
Open Access

TDP-43 Triggers Mitochondrial DNA Release via mPTP to Activate cGAS/STING in ALS.

Chien-Hsiung YuSeth L Masters
September 4, 2020
Open Access

C9orf72 poly(GR) aggregation induces TDP-43 proteinopathy.

Science Translational Medicine
Casey N CookLeonard Petrucelli

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