Developmental Neurobiology
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Rescue of polyglutamine-mediated cytotoxicity by double-stranded RNA-mediated RNA interference.
Human molecular genetics Jan, 2002 | Pubmed ID: 11809726
Toxic proteins in neurodegenerative disease.
Science (New York, N.Y.) Jun, 2002 | Pubmed ID: 12065827
Altered acetylation in polyglutamine disease: an opportunity for therapeutic intervention?
Trends in molecular medicine May, 2002 | Pubmed ID: 12067622
Hemolytic anemia presenting as idiopathic intracranial hypertension.
Neurology Sep, 2002 | Pubmed ID: 12297596
Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein.
Human molecular genetics Apr, 2003 | Pubmed ID: 12651870
Polyglutamines placed into context.
Neuron Jun, 2003 | Pubmed ID: 12797953
Aberrant histone acetylation, altered transcription, and retinal degeneration in a Drosophila model of polyglutamine disease are rescued by CREB-binding protein.
Genes & development Jun, 2003 | Pubmed ID: 12815067
Valproic acid increases SMN levels in spinal muscular atrophy patient cells.
Annals of neurology Nov, 2003 | Pubmed ID: 14595654
A screen for drugs that protect against the cytotoxicity of polyglutamine-expanded androgen receptor.
Human molecular genetics Feb, 2004 | Pubmed ID: 14709594
Hsp70 dynamics in vivo: effect of heat shock and protein aggregation.
Journal of cell science Oct, 2004 | Pubmed ID: 15367583
TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations.
Journal of neuropathology and experimental neurology Feb, 2007 | Pubmed ID: 17279000
Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy.
Acta neuropathologica Jul, 2007 | Pubmed ID: 17457594
Safety, tolerability, and pharmacokinetics of high-dose idebenone in patients with Friedreich ataxia.
Archives of neurology Jun, 2007 | Pubmed ID: 17562928
HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS.
Nature Jun, 2007 | Pubmed ID: 17568747
HDAC6 at the intersection of autophagy, the ubiquitin-proteasome system and neurodegeneration.
Autophagy Nov-Dec, 2007 | Pubmed ID: 17912024
The role of autophagy in age-related neurodegeneration.
Neuro-Signals , 2008 | Pubmed ID: 18097162
Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes.
Autophagy Feb, 2008 | Pubmed ID: 18188003
Autophagy and the ubiquitin-proteasome system: collaborators in neuroprotection.
Biochimica et biophysica acta Dec, 2008 | Pubmed ID: 18930136
Selective accumulation of aggregation-prone proteasome substrates in response to proteotoxic stress.
Molecular and cellular biology Apr, 2009 | Pubmed ID: 19158272
Polyglutamine-expanded androgen receptor truncation fragments activate a Bax-dependent apoptotic cascade mediated by DP5/Hrk.
The Journal of neuroscience : the official journal of the Society for Neuroscience Feb, 2009 | Pubmed ID: 19228953
Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis.
Muscle & nerve Jul, 2009 | Pubmed ID: 19533646
FOXO3a is broadly neuroprotective in vitro and in vivo against insults implicated in motor neuron diseases.
The Journal of neuroscience : the official journal of the Society for Neuroscience Jun, 2009 | Pubmed ID: 19553463
Overexpression of IGF-1 in muscle attenuates disease in a mouse model of spinal and bulbar muscular atrophy.
Neuron Aug, 2009 | Pubmed ID: 19679072
Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery.
Journal of proteome research Feb, 2010 | Pubmed ID: 20020773
Disease mutations in Rab7 result in unregulated nucleotide exchange and inappropriate activation.
Human molecular genetics Mar, 2010 | Pubmed ID: 20028791
HDAC6 controls autophagosome maturation essential for ubiquitin-selective quality-control autophagy.
The EMBO journal Mar, 2010 | Pubmed ID: 20075865
Dynein light chain 1 is required for autophagy, protein clearance, and cell death in Drosophila.
Proceedings of the National Academy of Sciences of the United States of America Jan, 2010 | Pubmed ID: 20080745
VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD.
Autophagy Feb, 2010 | Pubmed ID: 20104022
Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone.
Human molecular genetics May, 2010 | Pubmed ID: 20147319
Repeat expansion disease: progress and puzzles in disease pathogenesis.
Nature reviews. Genetics Apr, 2010 | Pubmed ID: 20177426
Flightless flies: Drosophila models of neuromuscular disease.
Annals of the New York Academy of Sciences Jan, 2010 | Pubmed ID: 20329357
B2 attenuates polyglutamine-expanded androgen receptor toxicity in cell and fly models of spinal and bulbar muscular atrophy.
Journal of neuroscience research Aug, 2010 | Pubmed ID: 20336775
Disease-causing mutations in parkin impair mitochondrial ubiquitination, aggregation, and HDAC6-dependent mitophagy.
The Journal of cell biology May, 2010 | Pubmed ID: 20457763
TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.
The Journal of neuroscience : the official journal of the Society for Neuroscience Jun, 2010 | Pubmed ID: 20519548
Native functions of the androgen receptor are essential to pathogenesis in a Drosophila model of spinobulbar muscular atrophy.
Neuron Sep, 2010 | Pubmed ID: 20869592
Exome sequencing reveals VCP mutations as a cause of familial ALS.
Neuron Dec, 2010 | Pubmed ID: 21145000
Histone deacetylases suppress CGG repeat-induced neurodegeneration via transcriptional silencing in models of fragile X tremor ataxia syndrome.
PLoS genetics , 2010 | Pubmed ID: 21170301
A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43.
Human molecular genetics Jul, 2011 | Pubmed ID: 21487023
Mutational analysis of the VCP gene in Parkinson's disease.
Neurobiology of aging Jan, 2012 | Pubmed ID: 21920633
Archetypal and New Families With Alexander Disease and Novel Mutations in GFAP.
Archives of neurology Feb, 2012 | Pubmed ID: 21987397
A novel conserved isoform of the ubiquitin ligase UFD2a/UBE4B is expressed exclusively in mature striated muscle cells.
PloS one , 2011 | Pubmed ID: 22174917