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Research
Member, St. Jude Faculty
Developmental Neurobiology
J. Paul Taylor, MD, PhD
Developmental Neurobiology
MS 343, D-4026
St. Jude Children's Research Hospital
262 Danny Thomas Place
Memphis, TN 38105-3678
Email: jpaul.taylor@stjude.org
Phone: (901) 595-6047
FAX: (901) 595-5947
MD PhD - Jefferson Medical College, Philadelphia, Pennsylvania
Neurology Residency – Hospital of the University of Pennsylvania
Neurogenetics Fellowship – Neurogenetics Branch, NINDS/NIH
The principal goal of our research is to reduce the morbidity and mortality associated with neurological disease. Toward this end we:
2013 Publications
Buchan JR*, Kolaitis R*, Taylor JP† and Parker R†. Eukaryotic stress granules are cleared by granulophagy and Cdc48/VCP function. Cell 2013 (in press). *co-first authors, †co-corresponding authors
Kim HJ, Kim NC, Wang M, Scarborough EA, Moore J, Diaz Z, Maclea K, Freibaum B, Molliex A, Kanagaraj A, Li S, Pinkus JL, Greenberg SA, Trojanowski J, Traynor BJ, Smith BN, Topp S, Gkazi AS, Miller J, Shaw CE, Wojtas AM, Boylan KB, Rademakers R, Kottlors M, Kirschner J, Li YR, Gitler AD, Benatar M, King OD, Kimonis V, Ross ED, Weihl CC, Shorter J, Taylor JP. Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS. Nature 495:467-473, 2013.
Taylor JP. RNA Gets RAN in Neurodegeneration Science 339:1282-1283, 2013.
Kim NC, Tresse E, Kolaitis RM, Molliex A, Thomas R, Alami N, Wang B, Joshi A, Smith RB, Ritson GP, Winborn BJ, Lee JY, Yao TP, Pallanck L, Kundu M, Taylor JP. VCP is essential for mitochondrial quality control by the PINK1/Parkin pathway and this function is impaired by disease-causing mutations. Neuron 78:65-80, 2013.
Benatar M, Weihl CC, Wuu J, Traynor B, Oskarsson B, Fernandez C, Taylor JP. Motor neuron involvement in multisystem proteinopathy: implications for ALS. Neurology 80(20):1874-1880, 2013.
Fujita K, Nakamura Y, Oka T, Ito H, Tarrua T, Tagawa K, Sasabe T, Katsuta A, Motoki K, Shiwaku H, Sone M, Yoshida C, Katsana M, Eishi Y, Murata M, Taylor JP, Wanker EE, Kono K, Tashiro S, Sobue G, La Spada AR, Okazawa H. A functional deficiency of TERA/VCP/p97 contributes to impaired DNA damage repair in multiple polyglutamine diseases. Nature Communications 4:1816, 2013. doi: 10.1038/ncomms2828.
Todd PK, Oh SY, Krans A, He F, Sellier C, Frazer M, Renoux AJ, Chen KC, Scaglione M, Basrur V, Elenitoba-Johnson K, Vonsattel JP, Louis ED, Sutton MA, Taylor JP, Mills RE, Charlet-Berguerand N, and Paulson HL. CGG Repeat-Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia Syndrome. Neuron 78(3):440-455, 2013.
2012 Publications
Gao FB, Taylor JP. RNA-binding proteins in neurological disease. Brain Research 1462:1-2. 2012.
Usenovic M, Tresse E, Taylor JP, Krainc D. Parkinsonism-linked ATP13A2 causes lysosomal dysfunction and α-synuclein accumulation. J Neurosci 32(12):4240-6. 2012.
Messing A, Li R, Naidu S, Taylor JP, van der Knaap MS, Flint D, Brenner M. Novel GFAP mutations in two archetypical Alexander disease families. Archives of Neurology 69(2):208-14. 2012.
Klionsky et al., Guidelines for the use and interpretation of assays for monitoring autophagy. Autophagy 8(4):445-544, 2012.
Abramzon Y, Johnson JO, Scholz SW, Taylor JP, Brunnetti M, Calvo A, Mandrioli J, Benatar M, Mora G, Restagno G, Chio A and Traynor BJ. Valosin-containing protein mutations in sporadic amyotrophic lateral sclerosis. Neurobiology of Aging 33(9):2231.e1-2231.e6. 2012.
Park YN, Zhao X, Norton M, Taylor JP, Eisenberg E and Greene LE. Huntingtin fragments and SOD1 mutants form soluble oligomers in the cell. PloS One 7(6):e40329, 2012.
2011 Publications
Smith RB, Taylor JP. Dissection and staining of active zones in the Drosophila neuromuscular junction. Journal of Visualized Experiments http://www.jove.com/video/2676/dissection-imaging-active-zones-drosophila-neuromuscular 2011.
Lanson N, Maltare A, King H, Smith R, Kim JH, Taylor JP, Lloyd TE and Pandey UB. A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43. Human Molecular Genetics 20(13):2510-23, 2011.
Majounie E, Traynor BT, Chio A, Restagno G, Benatar M, Taylor JP, Singleton AB. Mutational analysis of the VCP gene in Parkinson’s disease. Neurobiology of Aging 33(1): 209 2011.
Mammen, AL, Mahoney JA, St. Germain A, Badders N, Taylor JP, Rosen A, and Spinette S. A Novel Conserved Isoform of the Ubiquitin Ligase UFD2a/UBE4B is Expressed Exclusively in Mature Striated Muscle Cells. PloS One 6(12):e28861, 2011.
2010 Publications
Johnson JO, Mandrioli J, Benatar M, Abramzon Y, Van Deerlin VM, Trojanowski JQ, Gibbs JR, Brunetti M, Gronka S, Wuu J, Ding J, McCluskey L, Martinez-Lage M, Falcone D, Hernandezz DG, Arepelli S, Chong S, Schymick JC, Rothstein J, Landi F, Wang M, Calvo A, Mora G, Sabatelli M, Monsurro MR, Battaistini S, Salvi F, Spataro R, Sola P, Borghero G, ITALSGEN, Galassi G, Scholz SW, Taylor JP, Restagno G, Chio A, Traynor BJ. Exome sequencing reveals VCP mutations as a cause of familial ALS. Neuron 68(5):857-864, 2010.
Todd PK, Oh SY, Krans A, Pandey U, DiProspero N, Min KT, Taylor JP, and Paulson HL. Histone deacetylases suppress CGG repeat-induced neurodegeneration via transcriptional silencing in models of fragile X tremor ataxia syndrome. PLoS Genetics 6(12):1240, 2010.
Nedelsky NB, Pennuto M, Smith RB, Palazzolo I, Nie Z, Moore J, Neale G and Taylor JP. Native functions of the androgen receptor are essential to pathogenesis in a Drosophila model of spinobulbar muscular atrophy Neuron 67(6): 936-952, 2010.
Ritson GP, Custer SK, Freibaum BD, Guinto JB, Geffel D, Winton MJ, Neumann M, Trojanowski JQ, Lee VMY, Forman MS, Taylor JP. TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97. J Neurosci 30(22):7729-39, 2010.
Lee JY, Nagano Y, Taylor JP, Lim KL, Yao TP. Parkin controls mitophagy by promoting mitochondrial ubiquitination and aggresome-like mitochondrial aggregate formation. J Cell Biol 189(4):671-9, 2010.
La Spada AR, Taylor JP. Repeat expansion disease: progress and puzzles in disease pathogenesis. Nature Reviews Genetics 11, 247-256, 2010.
Batlevi Y, Martin DN, Pandey UB, Simon CR, Powers CM, Taylor JP, Baehrecke EB. (2010) Dynein light chain 1 is required for autophagy, protein clearance and cell death in Drosophila. Proc Nat Acad Sci USA 107:742-747, 2010.
Freibaum BD, Chitta R, High AA, Taylor JP. Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery. J Proteome Res 9:1104-1120, 2010.
Lee JY, Kawaguchi Y, Koga H, Tang W, Wong E, Pandey UB, Gao YS, Lu J, Taylor JP, Cuervo AM, Yao TP. HDAC6 controls autophagosome-lysosome fusion in basal homeostatic autophagy critical for protein aggregate clearance and neurodegeneration. EMBO J 29(5):969-980, 2010.
Tresse E, Salomons FA, Yao, TP, Dantuma NP, Taylor JP. VCP is essential for maturation of ubiquitin-containing autophagosomes. Autophagy 6, 217-227, 2010.
McCray BA, Skordalakes E, Taylor JP. Disease mutations in Rab7 result in unregulated nucleotide exchange and inappropriate activation. Human Molecular Genetics 19(6):1033-1047, 2010.
Custer SK, Neumann M, Lu H, Wright AC, Taylor JP. Transgenic mice expressing mutant forms of VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone. Human Molecular Genetics 19(9):1741-55, 2010.
Palazzolo I, Natalia Nedelsky NB, Askew C, Harmison G, Kasantsev AG, Taylor JP, Fischbeck KH, Pennuto M. B2 attenuates polyglutamine androgen receptor toxicity in spinal and bulbar muscular atrophy. J Neurosci Res 88(10):2207-16, 2010.
Lloyd T, Taylor JP. Flightless flies: Drosophila models of neuromuscular disease. Annals of the New York Acad Sci 1184:1-20, 2010.
Sample publications 2009 or earlier
Last update: May 2013
