Guillaume M. Hautbergue

385 Glossop Road
Sheffield, ENG, United Kingdom

Last Updated: 09/26/2026

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Found 54 publications

Antisense Dipeptide Repeat Proteins Drive Widescale Purine Metabolism Aberration in C9orf72 Amyotrophic Lateral Sclerosis via ADA.

Antisense Dipeptide Repeat Proteins Drive Widescale Purine Metabolism Aberration in C9orf72 Amyotrophic Lateral Sclerosis via ADA.

Journal: International journal of molecular sciences

Published: January 25, 2026

Comparison of AAV9-driven motor neuron transduction following different CNS-directed delivery methods in mice.

Comparison of AAV9-driven motor neuron transduction following different CNS-directed delivery methods in mice.

Journal: Scientific reports

Published: November 14, 2025

Amyotrophic lateral sclerosis caused by hexanucleotide repeat expansions in C9orf72: from genetics to therapeutics.

Amyotrophic lateral sclerosis caused by hexanucleotide repeat expansions in C9orf72: from genetics to therapeutics.

Journal: The Lancet. Neurology

Published: August 05, 2024

RNP granules in ALS and neurodegeneration: From multifunctional membraneless organelles to therapeutic opportunities.

RNP granules in ALS and neurodegeneration: From multifunctional membraneless organelles to therapeutic opportunities.

Journal: International review of neurobiology

Published: May 27, 2024

C9orf72 poly-PR forms anisotropic condensates causative of nuclear TDP-43 pathology.

C9orf72 poly-PR forms anisotropic condensates causative of nuclear TDP-43 pathology.

Journal: iScience

Published: April 11, 2024

RuvBL1/2 reduce toxic dipeptide repeat protein burden in multiple models of C9orf72-ALS/FTD.

RuvBL1/2 reduce toxic dipeptide repeat protein burden in multiple models of C9orf72-ALS/FTD.

Journal: Life science alliance

Published: April 05, 2024

RAN Translation of C9orf72-Related Dipeptide Repeat Proteins in Zebrafish Recapitulates Hallmarks of Amyotrophic Lateral Sclerosis and Identifies Hypothermia as a Therapeutic Strategy.

RAN Translation of C9orf72-Related Dipeptide Repeat Proteins in Zebrafish Recapitulates Hallmarks of Amyotrophic Lateral Sclerosis and Identifies Hypothermia as a Therapeutic Strategy.

Journal: Annals of neurology

Published: February 14, 2024

A cell-penetrant peptide blocking C9ORF72-repeat RNA nuclear export reduces the neurotoxic effects of dipeptide repeat proteins.

A cell-penetrant peptide blocking C9ORF72-repeat RNA nuclear export reduces the neurotoxic effects of dipeptide repeat proteins.

Journal: Science translational medicine

Published: March 01, 2023

A Y374X TDP43 truncation leads to an altered metabolic profile in amyotrophic lateral sclerosis fibroblasts driven by pyruvate and TCA cycle intermediate alterations.

A Y374X TDP43 truncation leads to an altered metabolic profile in amyotrophic lateral sclerosis fibroblasts driven by pyruvate and TCA cycle intermediate alterations.

Journal: Frontiers in aging neuroscience

Published: January 26, 2023

Loss of TMEM106B exacerbates C9ALS/FTD DPR pathology by disrupting autophagosome maturation.

Loss of TMEM106B exacerbates C9ALS/FTD DPR pathology by disrupting autophagosome maturation.

Journal: Frontiers in cellular neuroscience

Published: October 04, 2022

Low expression of EXOSC2 protects against clinical COVID-19 and impedes SARS-CoV-2 replication.

Low expression of EXOSC2 protects against clinical COVID-19 and impedes SARS-CoV-2 replication.

Journal: Life science alliance

Published: March 15, 2022

RNA Helicases in Microsatellite Repeat Expansion Disorders and Neurodegeneration.

RNA Helicases in Microsatellite Repeat Expansion Disorders and Neurodegeneration.

Journal: Frontiers in genetics

Published: February 28, 2022

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Last Updated: 09/26/2026