Adrian Higginbottom

385 Glossop Road
Sheffield, ENG, United Kingdom

Last Updated: 09/26/2026

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Found 39 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

A bacterial artificial chromosome mouse model of amyotrophic lateral sclerosis manifests 'space cadet syndrome' on two FVB backgrounds.

A bacterial artificial chromosome mouse model of amyotrophic lateral sclerosis manifests 'space cadet syndrome' on two FVB backgrounds.

Journal: Disease models & mechanisms
Published: April 16, 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

Pre-clinical development of AP4B1 gene replacement therapy for hereditary spastic paraplegia type 47.

Pre-clinical development of AP4B1 gene replacement therapy for hereditary spastic paraplegia type 47.

Journal: EMBO molecular medicine
Published: March 15, 2024

Co-aggregation with Apolipoprotein E modulates the function of Amyloid-β in Alzheimer's disease.

Co-aggregation with Apolipoprotein E modulates the function of Amyloid-β in Alzheimer's disease.

Journal: Nature communications
Published: February 28, 2024

Relationships of brain cholesterol and cholesterol biosynthetic enzymes to Alzheimer's pathology and dementia in the CFAS population-derived neuropathology cohort.

Relationships of brain cholesterol and cholesterol biosynthetic enzymes to Alzheimer's pathology and dementia in the CFAS population-derived neuropathology cohort.

Journal: Neuroscience research
Published: June 01, 2023

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

Prospects for gene replacement therapies in amyotrophic lateral sclerosis.

Prospects for gene replacement therapies in amyotrophic lateral sclerosis.

Journal: Nature reviews. Neurology
Published: November 10, 2022

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

SPG15 protein deficits are at the crossroads between lysosomal abnormalities, altered lipid metabolism and synaptic dysfunction.

SPG15 protein deficits are at the crossroads between lysosomal abnormalities, altered lipid metabolism and synaptic dysfunction.

Journal: Human molecular genetics
Published: January 09, 2022

Spatiotemporal Proteomic Analysis of Stress Granule Disassembly Using APEX Reveals Regulation by SUMOylation and Links to ALS Pathogenesis.

Spatiotemporal Proteomic Analysis of Stress Granule Disassembly Using APEX Reveals Regulation by SUMOylation and Links to ALS Pathogenesis.

Journal: Molecular cell
Published: January 31, 2020

Oligodendrocyte pathology exceeds axonal pathology in white matter in human amyotrophic lateral sclerosis.

Oligodendrocyte pathology exceeds axonal pathology in white matter in human amyotrophic lateral sclerosis.

Journal: The Journal of pathology
Published: December 06, 2019
Showing 1-12 of 39

Last Updated: 09/26/2026