List of works - J Paul Taylor

A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43.

scientific article

A PR plug for the nuclear pore in amyotrophic lateral sclerosis

scientific article published on 3 February 2017

A case of familial ALS due to multi-system proteinopathy 1 and Huntington disease

scientific article published on 10 September 2014

A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases

scientific article

A novel conserved isoform of the ubiquitin ligase UFD2a/UBE4B is expressed exclusively in mature striated muscle cells

scientific article

A screen for drugs that protect against the cytotoxicity of polyglutamine-expanded androgen receptor.

scientific article

A small-molecule Nrf1 and Nrf2 activator mitigates polyglutamine toxicity in spinal and bulbar muscular atrophy

scientific article

Aberrant histone acetylation, altered transcription, and retinal degeneration in a Drosophila model of polyglutamine disease are rescued by CREB-binding protein

scientific article

Abnormal distribution of heterogeneous nuclear ribonucleoproteins in sporadic inclusion body myositis

scientific article

Activation of expression of genes coding for extracellular matrix proteins in Tat-producing glioblastoma cells.

scientific article published on October 1992

Altered acetylation in polyglutamine disease: an opportunity for therapeutic intervention?

scientific article published on 01 May 2002

Altered ribostasis: RNA-protein granules in degenerative disorders

scientific article

Archetypal and new families with Alexander disease and novel mutations in GFAP.

scientific article published on 10 October 2011

Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutations

scientific article

Beyond aggregation: Pathological phase transitions in neurodegenerative disease

scientific article published on 01 October 2020

Bridging biophysics and neurology: aberrant phase transitions in neurodegenerative disease

scientific article published on 01 May 2019

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

scientific article published on October 2016

Cancer-associated DDX3X mutations drive stress granule assembly and impair global translation

scientific article

Chronic optogenetic induction of stress granules is cytotoxic and reveals the evolution of ALS-FTD pathology

scientific article published on 20 March 2019

Convergence of Parkin, PINK1, and α-Synuclein on Stress-induced Mitochondrial Morphological Remodeling

scientific article published on 10 April 2015

Decoding ALS: from genes to mechanism.

scientific article published on 9 November 2016

Dissection and imaging of active zones in the Drosophila neuromuscular junction.

scientific article

Effects of Mutations on the Aggregation Propensity of the Human Prion-Like Protein hnRNPA2B1.

scientific article published on 30 January 2017

Eukaryotic stress granules are cleared by autophagy and Cdc48/VCP function

scientific article

Exome sequencing reveals VCP mutations as a cause of familial ALS

scientific article

Fragile X protein mitigates TDP-43 toxicity by remodeling RNA granules and restoring translation

scientific article published on 18 September 2015

Genetic interaction of hnRNPA2B1 and DNAJB6 in a Drosophila model of multisystem proteinopathy

scientific article

Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery.

scientific article

HDAC6 controls autophagosome maturation essential for ubiquitin-selective quality-control autophagy

scientific article (publication date: 3 March 2010)

HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS

scientific article

Higher-order oligomerization promotes localization of SPOP to liquid nuclear speckles

scientific article published on 23 May 2016

Histone deacetylases suppress CGG repeat-induced neurodegeneration via transcriptional silencing in models of fragile X tremor ataxia syndrome.

scientific article

Hsp70 dynamics in vivo: effect of heat shock and protein aggregation.

scientific article published on 14 September 2004

Huntingtin Fragments and SOD1 Mutants Form Soluble Oligomers in the Cell ⬇️

scientific article published on June 29, 2012

Lost in Transportation: Nucleocytoplasmic Transport Defects in ALS and Other Neurodegenerative Diseases

scientific article

MFN1 deacetylation activates adaptive mitochondrial fusion and protects metabolically challenged mitochondria

scientific article

Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale.

scientific article published in April 2005

Motor neuron involvement in multisystem proteinopathy: implications for ALS

scientific article

Multisystem proteinopathy: Intersecting genetics in muscle, bone, and brain degeneration

scientific article

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

scientific article

Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis

scientific article (publication date: May 2014)

Native Functions of the Androgen Receptor Are Essential to Pathogenesis in a Drosophila Model of Spinobulbar Muscular Atrophy ⬇️

scientific article published on September 23, 2010

Network analyses reveal novel aspects of ALS pathogenesis

scientific article

Neurodegenerative diseases: G-quadruplex poses quadruple threat

scientific article published in Nature

Neuroscience. RNA that gets RAN in neurodegeneration.

scientific article published in March 2013

No mutations in hnRNPA1 and hnRNPA2B1 in Dutch patients with amyotrophic lateral sclerosis, frontotemporal dementia, and inclusion body myopathy

scientific article published on 6 February 2014

Novel mutations expand the clinical spectrum of DYNC1H1-associated spinal muscular atrophy

scientific article

Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics ⬇️

scientific article published on March 2017

Phase separation by low complexity domains promotes stress granule assembly and drives pathological fibrillization

scientific article

Profilin 1 associates with stress granules and ALS-linked mutations alter stress granule dynamics.

scientific article published in June 2014

Protein arginine methyltransferase 6 enhances polyglutamine-expanded androgen receptor function and toxicity in spinal and bulbar muscular atrophy.

scientific article

Protein-RNA Networks Regulated by Normal and ALS-Associated Mutant HNRNPA2B1 in the Nervous System

scientific article published on 13 October 2016

RAN translation at CGG repeats induces ubiquitin proteasome system impairment in models of fragile X-associated tremor ataxia syndrome

scientific article published on 7 May 2015

RNA metabolism in neurological disease.

scientific article published on October 2014

RNA-binding proteins in neurological disease ⬇️

scientific article published on June 26, 2012

Regulatory Role of RNA Chaperone TDP-43 for RNA Misfolding and Repeat-Associated Translation in SCA31.

scientific article published on 18 March 2017

Repeat expansion disease: progress and puzzles in disease pathogenesis

scientific article published on April 2010

Revealing the Mutational Spectrum in Southern Africans With Amyotrophic Lateral Sclerosis

scientific article published on 12 January 2022

Safety, Tolerability, and Pharmacokinetics of High-Dose Idebenone in Patients With Friedreich Ataxia

scientific article published on 01 June 2007

Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis

scientific article

Selective accumulation of aggregation-prone proteasome substrates in response to proteotoxic stress

scientific article

Sexual Reassignment Fails to Prevent Kennedy's Disease

scientific article published in March 2016

TDP-43 suppresses CGG repeat-induced neurotoxicity through interactions with HnRNP A2/B1.

scientific article

TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics

scientific article published in August 2017

Targeting protein homeostasis in sporadic inclusion body myositis

scientific article

The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD.

scientific article published on 13 February 2017

The role of autophagy in age-related neurodegeneration

scientific article

Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice

scientific article

VCP is essential for mitochondrial quality control by PINK1/Parkin and this function is impaired by VCP mutations

scientific article

VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD

scientific journal article

Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy

scientific article

Valproic acid increases SMN levels in spinal muscular atrophy patient cells.

scientific article published on November 2003

List of works by J Paul Taylor

A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43.

A PR plug for the nuclear pore in amyotrophic lateral sclerosis

A case of familial ALS due to multi-system proteinopathy 1 and Huntington disease

A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases

A novel conserved isoform of the ubiquitin ligase UFD2a/UBE4B is expressed exclusively in mature striated muscle cells

A screen for drugs that protect against the cytotoxicity of polyglutamine-expanded androgen receptor.

A small-molecule Nrf1 and Nrf2 activator mitigates polyglutamine toxicity in spinal and bulbar muscular atrophy

Aberrant histone acetylation, altered transcription, and retinal degeneration in a Drosophila model of polyglutamine disease are rescued by CREB-binding protein

Abnormal distribution of heterogeneous nuclear ribonucleoproteins in sporadic inclusion body myositis

Activation of expression of genes coding for extracellular matrix proteins in Tat-producing glioblastoma cells.

Altered acetylation in polyglutamine disease: an opportunity for therapeutic intervention?

Altered ribostasis: RNA-protein granules in degenerative disorders

Archetypal and new families with Alexander disease and novel mutations in GFAP.

Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutations

Beyond aggregation: Pathological phase transitions in neurodegenerative disease

Bridging biophysics and neurology: aberrant phase transitions in neurodegenerative disease

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

Cancer-associated DDX3X mutations drive stress granule assembly and impair global translation

Chronic optogenetic induction of stress granules is cytotoxic and reveals the evolution of ALS-FTD pathology

Convergence of Parkin, PINK1, and α-Synuclein on Stress-induced Mitochondrial Morphological Remodeling

Decoding ALS: from genes to mechanism.

Dissection and imaging of active zones in the Drosophila neuromuscular junction.

Effects of Mutations on the Aggregation Propensity of the Human Prion-Like Protein hnRNPA2B1.

Eukaryotic stress granules are cleared by autophagy and Cdc48/VCP function

Exome sequencing reveals VCP mutations as a cause of familial ALS

Fragile X protein mitigates TDP-43 toxicity by remodeling RNA granules and restoring translation

Genetic interaction of hnRNPA2B1 and DNAJB6 in a Drosophila model of multisystem proteinopathy

Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery.

HDAC6 controls autophagosome maturation essential for ubiquitin-selective quality-control autophagy

HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS

Higher-order oligomerization promotes localization of SPOP to liquid nuclear speckles

Histone deacetylases suppress CGG repeat-induced neurodegeneration via transcriptional silencing in models of fragile X tremor ataxia syndrome.

Hsp70 dynamics in vivo: effect of heat shock and protein aggregation.

Huntingtin Fragments and SOD1 Mutants Form Soluble Oligomers in the Cell ⬇️

Lost in Transportation: Nucleocytoplasmic Transport Defects in ALS and Other Neurodegenerative Diseases

MFN1 deacetylation activates adaptive mitochondrial fusion and protects metabolically challenged mitochondria

Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale.

Motor neuron involvement in multisystem proteinopathy: implications for ALS

Multisystem proteinopathy: Intersecting genetics in muscle, bone, and brain degeneration

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis

Native Functions of the Androgen Receptor Are Essential to Pathogenesis in a Drosophila Model of Spinobulbar Muscular Atrophy ⬇️

Network analyses reveal novel aspects of ALS pathogenesis

Neurodegenerative diseases: G-quadruplex poses quadruple threat

Neuroscience. RNA that gets RAN in neurodegeneration.

No mutations in hnRNPA1 and hnRNPA2B1 in Dutch patients with amyotrophic lateral sclerosis, frontotemporal dementia, and inclusion body myopathy

Novel mutations expand the clinical spectrum of DYNC1H1-associated spinal muscular atrophy

Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics ⬇️

Phase separation by low complexity domains promotes stress granule assembly and drives pathological fibrillization

Profilin 1 associates with stress granules and ALS-linked mutations alter stress granule dynamics.

Protein arginine methyltransferase 6 enhances polyglutamine-expanded androgen receptor function and toxicity in spinal and bulbar muscular atrophy.

Protein-RNA Networks Regulated by Normal and ALS-Associated Mutant HNRNPA2B1 in the Nervous System

RAN translation at CGG repeats induces ubiquitin proteasome system impairment in models of fragile X-associated tremor ataxia syndrome

RNA metabolism in neurological disease.

RNA-binding proteins in neurological disease ⬇️

Regulatory Role of RNA Chaperone TDP-43 for RNA Misfolding and Repeat-Associated Translation in SCA31.

Repeat expansion disease: progress and puzzles in disease pathogenesis

Revealing the Mutational Spectrum in Southern Africans With Amyotrophic Lateral Sclerosis

Safety, Tolerability, and Pharmacokinetics of High-Dose Idebenone in Patients With Friedreich Ataxia

Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis

Selective accumulation of aggregation-prone proteasome substrates in response to proteotoxic stress

Sexual Reassignment Fails to Prevent Kennedy's Disease

TDP-43 suppresses CGG repeat-induced neurotoxicity through interactions with HnRNP A2/B1.

TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics

Targeting protein homeostasis in sporadic inclusion body myositis

The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD.

The role of autophagy in age-related neurodegeneration

Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice

VCP is essential for mitochondrial quality control by PINK1/Parkin and this function is impaired by VCP mutations

VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD

Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy

Valproic acid increases SMN levels in spinal muscular atrophy patient cells.