J. Paul Taylor

Rescue of polyglutamine-mediated cytotoxicity by double-stranded RNA-mediated RNA interference.

Toxic proteins in neurodegenerative disease.

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

Hemolytic anemia presenting as idiopathic intracranial hypertension.

Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein.

Polyglutamines placed into context.

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

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

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

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

TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations.

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

Safety, tolerability, and pharmacokinetics of high-dose idebenone in patients with Friedreich ataxia.

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

HDAC6 at the intersection of autophagy, the ubiquitin-proteasome system and neurodegeneration.

The role of autophagy in age-related neurodegeneration.

Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes.

Autophagy and the ubiquitin-proteasome system: collaborators in neuroprotection.

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

Polyglutamine-expanded androgen receptor truncation fragments activate a Bax-dependent apoptotic cascade mediated by DP5/Hrk.

Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis.

FOXO3a is broadly neuroprotective in vitro and in vivo against insults implicated in motor neuron diseases.

Overexpression of IGF-1 in muscle attenuates disease in a mouse model of spinal and bulbar muscular atrophy.

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

Disease mutations in Rab7 result in unregulated nucleotide exchange and inappropriate activation.

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

Dynein light chain 1 is required for autophagy, protein clearance, and cell death in Drosophila.

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

Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone.

Repeat expansion disease: progress and puzzles in disease pathogenesis.

Flightless flies: Drosophila models of neuromuscular disease.

B2 attenuates polyglutamine-expanded androgen receptor toxicity in cell and fly models of spinal and bulbar muscular atrophy.

Disease-causing mutations in parkin impair mitochondrial ubiquitination, aggregation, and HDAC6-dependent mitophagy.

TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.

Native functions of the androgen receptor are essential to pathogenesis in a Drosophila model of spinobulbar muscular atrophy.

Exome sequencing reveals VCP mutations as a cause of familial ALS.

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

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

Mutational analysis of the VCP gene in Parkinson's disease.

Archetypal and New Families With Alexander Disease and Novel Mutations in GFAP.

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