Lista de obras - J Paul Taylor - Dominio Público Uruguay

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

artículo científico publicado en 2011

A PR plug for the nuclear pore in amyotrophic lateral sclerosis

artículo científico publicado en 2017

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

artículo científico publicado en 2014

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

artículo científico publicado en 2013

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

artículo científico publicado en 2011

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

artículo científico publicado en 2004

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

artículo científico publicado en 2016

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

artículo científico publicado en 2003

Abnormal distribution of heterogeneous nuclear ribonucleoproteins in sporadic inclusion body myositis

artículo científico publicado en 2014

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

artículo científico publicado en 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

artículo científico publicado en 2013

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

artículo científico publicado en 2011

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

artículo científico publicado en 2014

Beyond aggregation: Pathological phase transitions in neurodegenerative disease

artículo científico publicado en 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

artículo científico publicado en 2016

CGG Repeat-Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia Syndrome ⬇️

artículo científico publicado el 18 de abril de 2013

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

artículo científico publicado en 2016

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

artículo científico publicado en 2015

Decoding ALS: from genes to mechanism.

artículo científico publicado en 2016

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

artículo científico publicado en 2011

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

artículo científico publicado en 2017

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

artículo científico publicado en 2013

Exome sequencing reveals VCP mutations as a cause of familial ALS

artículo científico publicado en 2010

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

artículo científico publicado en 2015

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

artículo científico publicado en 2016

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

artículo científico publicado en 2010

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

artículo científico publicado en 2010

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

artículo científico publicado en 2007

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

artículo científico publicado en 2016

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

artículo científico publicado en 2010

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

artículo científico publicado en 2004

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

artículo científico publicado el 29 de junio de 2012

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

artículo científico publicado en 2017

MFN1 deacetylation activates adaptive mitochondrial fusion and protects metabolically challenged mitochondria

artículo científico publicado en 2014

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

artículo científico publicado en 2005

Motor neuron involvement in multisystem proteinopathy: implications for ALS

artículo científico publicado en 2013

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

artículo científico publicado en 2015

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

artículo científico publicado en 2013

Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis

artículo científico publicado en 2014

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

artículo científico publicado el 23 de septiembre de 2010

Network analyses reveal novel aspects of ALS pathogenesis

artículo científico publicado en 2015

Neurodegenerative diseases: G-quadruplex poses quadruple threat

scientific article published in Nature

Neuroscience. RNA that gets RAN in neurodegeneration.

artículo científico publicado en 2013

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

artículo científico publicado en 2014

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

artículo científico publicado en 2015

Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics ⬇️

artículo científico publicado en 2017

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

artículo científico publicado en 2015

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

artículo científico publicado en 2014

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

artículo científico publicado en 2015

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

artículo científico publicado en 2016

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

artículo científico publicado en 2015

RNA metabolism in neurological disease.

artículo científico publicado en 2014

RNA-binding proteins in neurological disease ⬇️

artículo científico publicado el 26 de junio de 2012

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

artículo científico publicado en 2017

Repeat expansion disease: progress and puzzles in disease pathogenesis

artículo científico publicado en 2010

Revealing the Mutational Spectrum in Southern Africans With Amyotrophic Lateral Sclerosis

artículo científico publicado en 2022

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

artículo científico publicado en 2007

Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis

artículo científico publicado en 2009

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

artículo científico publicado en 2009

Sexual Reassignment Fails to Prevent Kennedy's Disease

artículo científico publicado en 2016

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

artículo científico publicado en 2014

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

artículo científico publicado en 2017

Targeting protein homeostasis in sporadic inclusion body myositis

artículo científico publicado en 2016

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

artículo científico publicado en 2017

The role of autophagy in age-related neurodegeneration

artículo científico publicado en 2008

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

artículo científico publicado en 2017

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

artículo científico publicado en 2013

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

artículo científico publicado en 2007

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

artículo científico publicado en 2003

Lista de obras de 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

CGG Repeat-Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia Syndrome ⬇️

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.