MARIAN DIFIGLIA, PhD - Publications

Affiliations: 
MASS GENERAL INSTITUTE FOR NEURODEGENERATIVE DISEASE MASSACHUSSETS GENERAL HOSPITAL 
Area:
NEUROPATHOLOGY
Website:
http://www.mghmind.org/Faculty/difiglia_marian.html

139 high-probability publications. We are testing a new system for linking publications to authors. You can help! If you notice any inaccuracies, please sign in and mark papers as correct or incorrect matches. If you identify any major omissions or other inaccuracies in the publication list, please let us know.

Year Citation  Score
2016 Macakova M, Bohuslavova B, Vochozkova P, Pavlok A, Sedlackova M, Vidinska D, Vochyanova K, Liskova I, Valekova I, Baxa M, Ellederova Z, Klima J, Juhas S, Juhasova J, Klouckova J, ... ... DiFiglia M, et al. Mutated Huntingtin Causes Testicular Pathology in Transgenic Minipig Boars. Neuro-Degenerative Diseases. PMID 26959244 DOI: 10.1159/000443665  1
2015 Choudhury SR, Harris AF, Cabral DJ, Keeler AM, Sapp E, Ferreira JS, Gray-Edwards HL, Johnson JA, Johnson AK, Su Q, Stoica L, DiFiglia M, Aronin N, Martin DR, Gao G, et al. Widespread CNS gene transfer and silencing after systemic delivery of novel AAV-AS vector. Molecular Therapy : the Journal of the American Society of Gene Therapy. PMID 26708003 DOI: 10.1038/mt.2015.231  1
2015 Alterman JF, Hall LM, Coles AH, Hassler MR, Didiot MC, Chase K, Abraham J, Sottosanti E, Johnson E, Sapp E, Osborn MF, Difiglia M, Aronin N, Khvorova A. Hydrophobically Modified siRNAs Silence Huntingtin mRNA in Primary Neurons and Mouse Brain. Molecular Therapy. Nucleic Acids. 4: e266. PMID 26623938 DOI: 10.1038/mtna.2015.38  1
2015 Johnson E, Chase K, McGowan S, Mondo E, Pfister E, Mick E, Friedline RH, Kim JK, Sapp E, DiFiglia M, Aronin N. Safety of Striatal Infusion of siRNA in a Transgenic Huntington's Disease Mouse Model. Journal of Huntington's Disease. 4: 219-29. PMID 26444021 DOI: 10.3233/JHD-150163  1
2015 Vodicka P, Mo S, Tousley A, Green KM, Sapp E, Iuliano M, Sadri-Vakili G, Shaffer SA, Aronin N, DiFiglia M, Kegel-Gleason KB. Mass Spectrometry Analysis of Wild-Type and Knock-in Q140/Q140 Huntington's Disease Mouse Brains Reveals Changes in Glycerophospholipids Including Alterations in Phosphatidic Acid and Lyso-Phosphatidic Acid. Journal of Huntington's Disease. 4: 187-201. PMID 26397899 DOI: 10.3233/JHD-150149  1
2015 Yao Y, Cui X, Al-Ramahi I, Sun X, Li B, Hou J, Difiglia M, Palacino J, Wu ZY, Ma L, Botas J, Lu B. A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity. Elife. 4. PMID 25738228 DOI: 10.7554/eLife.05449  1
2014 Grishchuk Y, Sri S, Rudinskiy N, Ma W, Stember KG, Cottle MW, Sapp E, Difiglia M, Muzikansky A, Betensky RA, Wong AM, Bacskai BJ, Hyman BT, Kelleher RJ, Cooper JD, et al. Behavioral deficits, early gliosis, dysmyelination and synaptic dysfunction in a mouse model of mucolipidosis IV. Acta Neuropathologica Communications. 2: 133. PMID 25200117 DOI: 10.1186/s40478-014-0133-7  1
2014 Aronin N, DiFiglia M. Huntingtin-lowering strategies in Huntington's disease: antisense oligonucleotides, small RNAs, and gene editing. Movement Disorders : Official Journal of the Movement Disorder Society. 29: 1455-61. PMID 25164989 DOI: 10.1002/mds.26020  1
2014 Vodicka P, Lim J, Williams DT, Kegel KB, Chase K, Park H, Marchionini D, Wilkinson S, Mead T, Birch H, Yates D, Lyons K, Dominguez C, Beconi M, Yue Z, ... ... DiFiglia M, et al. Assessment of chloroquine treatment for modulating autophagy flux in brain of WT and HD mice. Journal of Huntington's Disease. 3: 159-74. PMID 25062859 DOI: 10.3233/JHD-130081  1
2014 Sheng R, Zhang TT, Felice VD, Qin T, Qin ZH, Smith CD, Sapp E, Difiglia M, Waeber C. Preconditioning stimuli induce autophagy via sphingosine kinase 2 in mouse cortical neurons. The Journal of Biological Chemistry. 289: 20845-57. PMID 24928515 DOI: 10.1074/jbc.M114.578120  1
2014 Yu S, Liang Y, Palacino J, Difiglia M, Lu B. Drugging unconventional targets: insights from Huntington's disease. Trends in Pharmacological Sciences. 35: 53-62. PMID 24388390 DOI: 10.1016/j.tips.2013.12.001  1
2013 Baxa M, Hruska-Plochan M, Juhas S, Vodicka P, Pavlok A, Juhasova J, Miyanohara A, Nejime T, Klima J, Macakova M, Marsala S, Weiss A, Kubickova S, Musilova P, Vrtel R, ... ... DiFiglia M, et al. A transgenic minipig model of Huntington's Disease. Journal of Huntington's Disease. 2: 47-68. PMID 25063429 DOI: 10.3233/JHD-130001  1
2013 Liu W, Chaurette J, Pfister EL, Kennington LA, Chase KO, Bullock J, Vonsattel JP, Faull RL, Macdonald D, DiFiglia M, Zamore PD, Aronin N. Increased Steady-State Mutant Huntingtin mRNA in Huntington's Disease Brain. Journal of Huntington's Disease. 2: 491-500. PMID 25062733 DOI: 10.3233/JHD-130079  1
2013 Valencia A, Sapp E, Kimm JS, McClory H, Ansong KA, Yohrling G, Kwak S, Kegel KB, Green KM, Shaffer SA, Aronin N, DiFiglia M. Striatal synaptosomes from Hdh140Q/140Q knock-in mice have altered protein levels, novel sites of methionine oxidation, and excess glutamate release after stimulation. Journal of Huntington's Disease. 2: 459-75. PMID 24696705 DOI: 10.3233/JHD-130080  1
2013 O'Rourke JG, Gareau JR, Ochaba J, Song W, Raskó T, Reverter D, Lee J, Monteys AM, Pallos J, Mee L, Vashishtha M, Apostol BL, Nicholson TP, Illes K, Zhu YZ, ... ... Difiglia M, et al. SUMO-2 and PIAS1 modulate insoluble mutant huntingtin protein accumulation. Cell Reports. 4: 362-75. PMID 23871671 DOI: 10.1016/j.celrep.2013.06.034  1
2013 Lu B, Al-Ramahi I, Valencia A, Wang Q, Berenshteyn F, Yang H, Gallego-Flores T, Ichcho S, Lacoste A, Hild M, Difiglia M, Botas J, Palacino J. Identification of NUB1 as a suppressor of mutant Huntington toxicity via enhanced protein clearance. Nature Neuroscience. 16: 562-70. PMID 23525043 DOI: 10.1038/nn.3367  1
2013 Valencia A, Sapp E, Kimm JS, McClory H, Reeves PB, Alexander J, Ansong KA, Masso N, Frosch MP, Kegel KB, Li X, DiFiglia M. Elevated NADPH oxidase activity contributes to oxidative stress and cell death in Huntington's disease. Human Molecular Genetics. 22: 1112-31. PMID 23223017 DOI: 10.1093/hmg/dds516  1
2012 Qi L, Zhang XD, Wu JC, Lin F, Wang J, DiFiglia M, Qin ZH. The role of chaperone-mediated autophagy in huntingtin degradation. Plos One. 7: e46834. PMID 23071649 DOI: 10.1371/journal.pone.0046834  1
2012 Wu JC, Qi L, Wang Y, Kegel KB, Yoder J, Difiglia M, Qin ZH, Lin F. The regulation of N-terminal Huntingtin (Htt552) accumulation by Beclin1. Acta Pharmacologica Sinica. 33: 743-51. PMID 22543707 DOI: 10.1038/aps.2012.14  1
2012 Li X, Valencia A, McClory H, Sapp E, Kegel KB, Difiglia M. Deficient Rab11 activity underlies glucose hypometabolism in primary neurons of Huntington's disease mice. Biochemical and Biophysical Research Communications. 421: 727-30. PMID 22542623 DOI: 10.1016/j.bbrc.2012.04.070  1
2012 Ritch JJ, Valencia A, Alexander J, Sapp E, Gatune L, Sangrey GR, Sinha S, Scherber CM, Zeitlin S, Sadri-Vakili G, Irimia D, Difiglia M, Kegel KB. Multiple phenotypes in Huntington disease mouse neural stem cells. Molecular and Cellular Neurosciences. 50: 70-81. PMID 22508027 DOI: 10.1016/j.mcn.2012.03.011  1
2012 Wang L, Lin F, Wang J, Wu J, Han R, Zhu L, Difiglia M, Qin Z. Expression of mutant N-terminal huntingtin fragment (htt552-100Q) in astrocytes suppresses the secretion of BDNF. Brain Research. 1449: 69-82. PMID 22410294 DOI: 10.1016/j.brainres.2012.01.077  1
2012 Sapp E, Valencia A, Li X, Aronin N, Kegel KB, Vonsattel JP, Young AB, Wexler N, DiFiglia M. Native mutant huntingtin in human brain: evidence for prevalence of full-length monomer. The Journal of Biological Chemistry. 287: 13487-99. PMID 22375012 DOI: 10.1074/jbc.M111.286609  1
2012 Chen LL, Wu JC, Wang LH, Wang J, Qin ZH, Difiglia M, Lin F. Rapamycin prevents the mutant huntingtin-suppressed GLT-1 expression in cultured astrocytes. Acta Pharmacologica Sinica. 33: 385-92. PMID 22266730 DOI: 10.1038/aps.2011.162  1
2012 Wang L, Lin F, Wang J, Wu J, Han R, Zhu L, Zhang G, DiFiglia M, Qin Z. Truncated N-terminal huntingtin fragment with expanded-polyglutamine (htt552-100Q) suppresses brain-derived neurotrophic factor transcription in astrocytes. Acta Biochimica Et Biophysica Sinica. 44: 249-58. PMID 22234237 DOI: 10.1093/abbs/gmr125  1
2012 Valencia A, Sapp E, Reeves PB, Alexander J, Masso N, Li X, Kegel KB, DiFiglia M. Reagents that block neuronal death from Huntington's disease also curb oxidative stress. Neuroreport. 23: 10-5. PMID 22045254 DOI: 10.1097/WNR.0b013e32834d92e6  1
2012 Li X, DiFiglia M. The recycling endosome and its role in neurological disorders. Progress in Neurobiology. 97: 127-41. PMID 22037413 DOI: 10.1016/j.pneurobio.2011.10.002  1
2011 Fox JH, Connor T, Stiles M, Kama J, Lu Z, Dorsey K, Lieberman G, Liebermann G, Sapp E, Cherny RA, Banks M, Volitakis I, DiFiglia M, Berezovska O, Bush AI, et al. Cysteine oxidation within N-terminal mutant huntingtin promotes oligomerization and delays clearance of soluble protein. The Journal of Biological Chemistry. 286: 18320-30. PMID 21454633 DOI: 10.1074/jbc.M110.199448  1
2011 Fox JH, Connor T, Stiles M, Kama J, Lu Z, Dorsey K, Lieberman G, Sapp E, Cherny RA, Banks M, Volitakis I, DiFiglia M, Berezovska O, Bush AI, Hersch SM. Cysteine oxidation within N-terminal mutant huntingtin promotes oligomerization and delays clearance of soluble protein (Journal of Biological Chemistry (2011) 286, (18320-18330)) Journal of Biological Chemistry. 286: 27068. DOI: 10.1074/jbc.A110.199448  1
2010 Kegel KB, Sapp E, Alexander J, Reeves P, Bleckmann D, Sobin L, Masso N, Valencia A, Jeong H, Krainc D, Palacino J, Curtis D, Kuhn R, Betschart C, Sena-Esteves M, ... ... Difiglia M, et al. Huntingtin cleavage product A forms in neurons and is reduced by gamma-secretase inhibitors. Molecular Neurodegeneration. 5: 58. PMID 21156064 DOI: 10.1186/1750-1326-5-58  1
2010 Fox JH, Connor T, Chopra V, Dorsey K, Kama JA, Bleckmann D, Betschart C, Hoyer D, Frentzel S, Difiglia M, Paganetti P, Hersch SM. The mTOR kinase inhibitor Everolimus decreases S6 kinase phosphorylation but fails to reduce mutant huntingtin levels in brain and is not neuroprotective in the R6/2 mouse model of Huntington's disease. Molecular Neurodegeneration. 5: 26. PMID 20569486 DOI: 10.1186/1750-1326-5-26  1
2010 Li X, Valencia A, Sapp E, Masso N, Alexander J, Reeves P, Kegel KB, Aronin N, Difiglia M. Aberrant Rab11-dependent trafficking of the neuronal glutamate transporter EAAC1 causes oxidative stress and cell death in Huntington's disease. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 30: 4552-61. PMID 20357106 DOI: 10.1523/JNEUROSCI.5865-09.2010  1
2010 Legleiter J, Mitchell E, Lotz GP, Sapp E, Ng C, DiFiglia M, Thompson LM, Muchowski PJ. Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo. The Journal of Biological Chemistry. 285: 14777-90. PMID 20220138 DOI: 10.1074/jbc.M109.093708  1
2010 Valencia A, Reeves PB, Sapp E, Li X, Alexander J, Kegel KB, Chase K, Aronin N, DiFiglia M. Mutant huntingtin and glycogen synthase kinase 3-beta accumulate in neuronal lipid rafts of a presymptomatic knock-in mouse model of Huntington's disease. Journal of Neuroscience Research. 88: 179-90. PMID 19642201 DOI: 10.1002/jnr.22184  1
2009 Li X, Standley C, Sapp E, Valencia A, Qin ZH, Kegel KB, Yoder J, Comer-Tierney LA, Esteves M, Chase K, Alexander J, Masso N, Sobin L, Bellve K, Tuft R, ... ... DiFiglia M, et al. Mutant huntingtin impairs vesicle formation from recycling endosomes by interfering with Rab11 activity. Molecular and Cellular Biology. 29: 6106-16. PMID 19752198 DOI: 10.1128/MCB.00420-09  1
2009 Li X, Sapp E, Chase K, Comer-Tierney LA, Masso N, Alexander J, Reeves P, Kegel KB, Valencia A, Esteves M, Aronin N, Difiglia M. Disruption of Rab11 activity in a knock-in mouse model of Huntington's disease. Neurobiology of Disease. 36: 374-83. PMID 19699304 DOI: 10.1016/j.nbd.2009.08.003  1
2009 Weiss A, Abramowski D, Bibel M, Bodner R, Chopra V, DiFiglia M, Fox J, Kegel K, Klein C, Grueninger S, Hersch S, Housman D, Régulier E, Rosas HD, Stefani M, et al. Single-step detection of mutant huntingtin in animal and human tissues: a bioassay for Huntington's disease. Analytical Biochemistry. 395: 8-15. PMID 19664996 DOI: 10.1016/j.ab.2009.08.001  1
2009 Kegel KB, Schewkunow V, Sapp E, Masso N, Wanker EE, DiFiglia M, Goldmann WH. Polyglutamine expansion in huntingtin increases its insertion into lipid bilayers. Biochemical and Biophysical Research Communications. 387: 472-5. PMID 19607813 DOI: 10.1016/j.bbrc.2009.07.039  1
2009 Kegel KB, Sapp E, Alexander J, Valencia A, Reeves P, Li X, Masso N, Sobin L, Aronin N, DiFiglia M. Polyglutamine expansion in huntingtin alters its interaction with phospholipids. Journal of Neurochemistry. 110: 1585-97. PMID 19566678 DOI: 10.1111/j.1471-4159.2009.06255.x  1
2009 Pfister EL, Kennington L, Straubhaar J, Wagh S, Liu W, DiFiglia M, Landwehrmeyer B, Vonsattel JP, Zamore PD, Aronin N. Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients. Current Biology : Cb. 19: 774-8. PMID 19361997 DOI: 10.1016/j.cub.2009.03.030  1
2008 Li X, Sapp E, Valencia A, Kegel KB, Qin ZH, Alexander J, Masso N, Reeves P, Ritch JJ, Zeitlin S, Aronin N, Difiglia M. A function of huntingtin in guanine nucleotide exchange on Rab11. Neuroreport. 19: 1643-7. PMID 18845944 DOI: 10.1097/WNR.0b013e328315cd4c  1
2008 Klionsky DJ, Abeliovich H, Agostinis P, Agrawal DK, Aliev G, Askew DS, Baba M, Baehrecke EH, Bahr BA, Ballabio A, Bamber BA, Bassham DC, Bergamini E, Bi X, Biard-Piechaczyk M, ... ... Difiglia M, et al. Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes. Autophagy. 4: 151-75. PMID 18188003  1
2007 DiFiglia M, Sena-Esteves M, Chase K, Sapp E, Pfister E, Sass M, Yoder J, Reeves P, Pandey RK, Rajeev KG, Manoharan M, Sah DW, Zamore PD, Aronin N. Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits. Proceedings of the National Academy of Sciences of the United States of America. 104: 17204-9. PMID 17940007 DOI: 10.1073/pnas.0708285104  1
2006 Kim YJ, Sapp E, Cuiffo BG, Sobin L, Yoder J, Kegel KB, Qin ZH, Detloff P, Aronin N, DiFiglia M. Lysosomal proteases are involved in generation of N-terminal huntingtin fragments. Neurobiology of Disease. 22: 346-56. PMID 16423528 DOI: 10.1016/j.nbd.2005.11.012  1
2005 Rubinsztein DC, DiFiglia M, Heintz N, Nixon RA, Qin ZH, Ravikumar B, Stefanis L, Tolkovsky A. Autophagy and its possible roles in nervous system diseases, damage and repair. Autophagy. 1: 11-22. PMID 16874045  1
2005 Kalanithi PS, Zheng W, Kataoka Y, DiFiglia M, Grantz H, Saper CB, Schwartz ML, Leckman JF, Vaccarino FM. Altered parvalbumin-positive neuron distribution in basal ganglia of individuals with Tourette syndrome. Proceedings of the National Academy of Sciences of the United States of America. 102: 13307-12. PMID 16131542 DOI: 10.1073/pnas.0502624102  1
2005 Kegel KB, Sapp E, Yoder J, Cuiffo B, Sobin L, Kim YJ, Qin ZH, Hayden MR, Aronin N, Scott DL, Isenberg G, Goldmann WH, DiFiglia M. Huntingtin associates with acidic phospholipids at the plasma membrane. The Journal of Biological Chemistry. 280: 36464-73. PMID 16085648 DOI: 10.1074/jbc.M503672200  1
2005 Liang ZQ, Wang XX, Wang Y, Chuang DM, DiFiglia M, Chase TN, Qin ZH. Susceptibility of striatal neurons to excitotoxic injury correlates with basal levels of Bcl-2 and the induction of P53 and c-Myc immunoreactivity. Neurobiology of Disease. 20: 562-73. PMID 15922606 DOI: 10.1016/j.nbd.2005.04.011  1
2005 Ariano MA, Cepeda C, Calvert CR, Flores-Hernández J, Hernández-Echeagaray E, Klapstein GJ, Chandler SH, Aronin N, DiFiglia M, Levine MS. Striatal potassium channel dysfunction in Huntington's disease transgenic mice. Journal of Neurophysiology. 93: 2565-74. PMID 15625098 DOI: 10.1152/jn.00791.2004  1
2004 Qin ZH, Wang Y, Sapp E, Cuiffo B, Wanker E, Hayden MR, Kegel KB, Aronin N, DiFiglia M. Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 24: 269-81. PMID 14715959 DOI: 10.1523/JNEUROSCI.1409-03.2004  1
2003 Qin ZH, Wang Y, Kegel KB, Kazantsev A, Apostol BL, Thompson LM, Yoder J, Aronin N, DiFiglia M. Autophagy regulates the processing of amino terminal huntingtin fragments. Human Molecular Genetics. 12: 3231-44. PMID 14570716 DOI: 10.1093/hmg/ddg346  1
2003 Kim M, Roh JK, Yoon BW, Kang L, Kim YJ, Aronin N, DiFiglia M. Huntingtin is degraded to small fragments by calpain after ischemic injury. Experimental Neurology. 183: 109-15. PMID 12957494 DOI: 10.1016/S0014-4886(03)00132-8  1
2002 DiFiglia M. Huntingtin fragments that aggregate go their separate ways. Molecular Cell. 10: 224-5. PMID 12191468 DOI: 10.1016/S1097-2765(02)00609-3  1
2002 Chan EY, Luthi-Carter R, Strand A, Solano SM, Hanson SA, DeJohn MM, Kooperberg C, Chase KO, DiFiglia M, Young AB, Leavitt BR, Cha JH, Aronin N, Hayden MR, Olson JM. Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease. Human Molecular Genetics. 11: 1939-51. PMID 12165556  1
2002 Ariano MA, Aronin N, Difiglia M, Tagle DA, Sibley DR, Leavitt BR, Hayden MR, Levine MS. Striatal neurochemical changes in transgenic models of Huntington's disease. Journal of Neuroscience Research. 68: 716-29. PMID 12111832 DOI: 10.1002/jnr.10272  1
2002 Petersén A, Chase K, Puschban Z, DiFiglia M, Brundin P, Aronin N. Maintenance of susceptibility to neurodegeneration following intrastriatal injections of quinolinic acid in a new transgenic mouse model of Huntington's disease. Experimental Neurology. 175: 297-300. PMID 12009780 DOI: 10.1006/exnr.2002.7885  1
2002 Kegel KB, Meloni AR, Yi Y, Kim YJ, Doyle E, Cuiffo BG, Sapp E, Wang Y, Qin ZH, Chen JD, Nevins JR, Aronin N, DiFiglia M. Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription. The Journal of Biological Chemistry. 277: 7466-76. PMID 11739372 DOI: 10.1074/jbc.M103946200  1
2001 Petersén A, Hansson O, Puschban Z, Sapp E, Romero N, Castilho RF, Sulzer D, Rice M, DiFiglia M, Przedborski S, Brundin P. Mice transgenic for exon 1 of the Huntington's disease gene display reduced striatal sensitivity to neurotoxicity induced by dopamine and 6-hydroxydopamine. The European Journal of Neuroscience. 14: 1425-35. PMID 11722604 DOI: 10.1046/j.0953-816X.2001.01765.x  1
2001 Laforet GA, Sapp E, Chase K, McIntyre C, Boyce FM, Campbell M, Cadigan BA, Warzecki L, Tagle DA, Reddy PH, Cepeda C, Calvert CR, Jokel ES, Klapstein GJ, Ariano MA, ... ... DiFiglia M, et al. Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 21: 9112-23. PMID 11717344  1
2001 Kim YJ, Yi Y, Sapp E, Wang Y, Cuiffo B, Kegel KB, Qin ZH, Aronin N, DiFiglia M. Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis. Proceedings of the National Academy of Sciences of the United States of America. 98: 12784-9. PMID 11675509 DOI: 10.1073/pnas.221451398  1
2001 Sapp E, Kegel KB, Aronin N, Hashikawa T, Uchiyama Y, Tohyama K, Bhide PG, Vonsattel JP, DiFiglia M. Early and progressive accumulation of reactive microglia in the Huntington disease brain. Journal of Neuropathology and Experimental Neurology. 60: 161-72. PMID 11273004  1
2001 Qin ZH, Wang Y, Kikly KK, Sapp E, Kegel KB, Aronin N, DiFiglia M. Pro-caspase-8 is predominantly localized in mitochondria and released into cytoplasm upon apoptotic stimulation. The Journal of Biological Chemistry. 276: 8079-86. PMID 11102441 DOI: 10.1074/jbc.M007028200  1
2000 Kegel KB, Kim M, Sapp E, McIntyre C, Castaño JG, Aronin N, DiFiglia M. Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 20: 7268-78. PMID 11007884  1
2000 DiFiglia M. Genetics of childhood disorders: X. Huntington disease. Journal of the American Academy of Child and Adolescent Psychiatry. 39: 120-2. PMID 10638076  1
1999 Aronin N, Kim M, Laforet G, DiFiglia M. Are there multiple pathways in the pathogenesis of Huntington's disease? Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences. 354: 995-1003. PMID 10434298 DOI: 10.1098/rstb.1999.0451  1
1999 Kim M, Velier J, Chase K, Laforet G, Kalchman MA, Hayden MR, Won L, Heller A, Aronin N, Difiglia M. Forskolin and dopamine D1 receptor activation increase huntingtin's association with endosomes in immortalized neuronal cells of striatal origin. Neuroscience. 89: 1159-67. PMID 10362304 DOI: 10.1016/S0306-4522(98)00400-X  1
1999 Sapp E, Penney J, Young A, Aronin N, Vonsattel JP, DiFiglia M. Axonal transport of N-terminal huntingtin suggests early pathology of corticostriatal projections in Huntington disease. Journal of Neuropathology and Experimental Neurology. 58: 165-73. PMID 10029099  1
1999 Kim M, Lee HS, LaForet G, McIntyre C, Martin EJ, Chang P, Kim TW, Williams M, Reddy PH, Tagle D, Boyce FM, Won L, Heller A, Aronin N, DiFiglia M. Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 19: 964-73. PMID 9920660  1
1999 Martin EJ, Kim M, Velier J, Sapp E, Lee HS, Laforet G, Won L, Chase K, Bhide PG, Heller A, Aronin N, Difiglia M. Analysis of Huntingtin-associated protein 1 in mouse brain and immortalized striatal neurons. The Journal of Comparative Neurology. 403: 421-30. PMID 9888310 DOI: 10.1002/(SICI)1096-9861(19990125)403:4<421::AID-CNE1>3.0.CO;2-5  1
1998 Velier J, Kim M, Schwarz C, Kim TW, Sapp E, Chase K, Aronin N, DiFiglia M. Wild-type and mutant huntingtins function in vesicle trafficking in the secretory and endocytic pathways. Experimental Neurology. 152: 34-40. PMID 9682010 DOI: 10.1006/exnr.1998.6832  1
1998 Vonsattel JP, DiFiglia M. Huntington disease. Journal of Neuropathology and Experimental Neurology. 57: 369-84. PMID 9596408  1
1998 Davies SW, Beardsall K, Turmaine M, DiFiglia M, Aronin N, Bates GP. Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions? Lancet (London, England). 351: 131-3. PMID 9439509 DOI: 10.1016/S0140-6736(97)08360-8  1
1997 Sapp E, Schwarz C, Chase K, Bhide PG, Young AB, Penney J, Vonsattel JP, Aronin N, DiFiglia M. Huntingtin localization in brains of normal and Huntington's disease patients. Annals of Neurology. 42: 604-12. PMID 9382472 DOI: 10.1002/ana.410420411  1
1997 DiFiglia M, Sapp E, Chase KO, Davies SW, Bates GP, Vonsattel JP, Aronin N. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science (New York, N.Y.). 277: 1990-3. PMID 9302293 DOI: 10.1126/science.277.5334.1990  1
1997 Davies SW, Turmaine M, Cozens BA, DiFiglia M, Sharp AH, Ross CA, Scherzinger E, Wanker EE, Mangiarini L, Bates GP. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell. 90: 537-48. PMID 9267033 DOI: 10.1016/S0092-8674(00)80513-9  1
1997 DiFiglia M. Clinical Genetics, II. Huntington's disease: from the gene to pathophysiology. The American Journal of Psychiatry. 154: 1046. PMID 9247386  1
1997 Block-Galarza J, Chase KO, Sapp E, Vaughn KT, Vallee RB, DiFiglia M, Aronin N. Fast transport and retrograde movement of huntingtin and HAP 1 in axons. Neuroreport. 8: 2247-51. PMID 9243620  1
1996 Bhide PG, Day M, Sapp E, Schwarz C, Sheth A, Kim J, Young AB, Penney J, Golden J, Aronin N, DiFiglia M. Expression of normal and mutant huntingtin in the developing brain. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 16: 5523-35. PMID 8757264  1
1996 Reeves SA, Ueki K, Sinha B, Difiglia M, Louis DN. Regional expression and subcellular localization of the tyrosine-specific phosphatase SH-PTP2 in the adult human nervous system. Neuroscience. 71: 1037-42. PMID 8684607 DOI: 10.1016/0306-4522(95)00491-2  1
1995 Kanai Y, Bhide PG, DiFiglia M, Hediger MA. Neuronal high-affinity glutamate transport in the rat central nervous system. Neuroreport. 6: 2357-62. PMID 8747153  1
1995 Landwehrmeyer GB, McNeil SM, Dure IV LS, Ge P, Aizawa H, Huang Q, Ambrose CM, Duyao MP, Bird ED, Bonilla E, De Young M, Avila-Gonzales AJ, Wexler NS, DiFiglia M, Gusella JF, et al. Huntington's disease gene: Regional and cellular expression in brain of normal and affected individuals Annals of Neurology. 37: 218-230. PMID 7847863 DOI: 10.1002/ana.410370213  1
1995 Vonsattel JP, Aizawa H, Ge P, DiFiglia M, McKee AC, MacDonald M, Gusella JF, Landwehrmeyer GB, Bird ED, Richardson EP. An improved approach to prepare human brains for research. Journal of Neuropathology and Experimental Neurology. 54: 42-56. PMID 7815079  1
1995 Huang Q, Zhou D, Sapp E, Aizawa H, Ge P, Bird ED, Vonsattel JP, DiFiglia M. Quinolinic acid-induced increases in calbindin D28k immunoreactivity in rat striatal neurons in vivo and in vitro mimic the pattern seen in Huntington's disease. Neuroscience. 65: 397-407. PMID 7777157 DOI: 10.1016/0306-4522(94)00494-P  1
1995 DiFiglia M, Sapp E, Chase K, Schwarz C, Meloni A, Young C, Martin E, Vonsattel JP, Carraway R, Reeves SA. Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons. Neuron. 14: 1075-81. PMID 7748555 DOI: 10.1016/0896-6273(95)90346-1  1
1995 Aronin N, Chase K, Young C, Sapp E, Schwarz C, Matta N, Kornreich R, Lanwehrmeyer B, Bird E, Beal MF, Vonsattel JP, Smith T, Carraway R, Boyce FM, Young AB, ... ... DiFiglia M, et al. CAG expansion affects the expression of mutant huntingtin in the Huntington's disease brain Neuron. 15: 1193-1201. PMID 7576661 DOI: 10.1016/0896-6273(95)90106-X  1
1995 Sapp E, Ge P, Aizawa H, Bird E, Penney J, Young AB, Vonsattel JP, DiFiglia M. Evidence for a preferential loss of enkephalin immunoreactivity in the external globus pallidus in low grade Huntington's disease using high resolution image analysis. Neuroscience. 64: 397-404. PMID 7535402 DOI: 10.1016/0306-4522(94)00427-7  1
1994 Dure IV LS, Bernhard Landwehrmeyer G, Golden J, McNeil SM, Ge P, Aizawa H, Huang Q, Ambrose CM, Duyao MP, Bird ED, DiFiglia M, Gusella JF, MacDonald ME, Penney JB, Young AB, et al. IT15 gene expression in fetal human brain Brain Research. 659: 33-41. PMID 7820679 DOI: 10.1016/0006-8993(94)90860-5  1
1993 Cole DG, Growdon JH, DiFiglia M. Levodopa induction of Fos immunoreactivity in rat brain following partial and complete lesions of the substantia nigra. Experimental Neurology. 120: 223-32. PMID 8491280 DOI: 10.1006/exnr.1993.1057  1
1993 Zhou D, DiFiglia M. Basic fibroblast growth factor enhances the growth of postnatal neostriatal GABAergic neurons in vitro. Experimental Neurology. 122: 171-88. PMID 8405257 DOI: 10.1006/exnr.1993.1118  1
1993 Roberts RC, Ahn A, Swartz KJ, Beal MF, DiFiglia M. Intrastriatal injections of quinolinic acid or kainic acid: differential patterns of cell survival and the effects of data analysis on outcome. Experimental Neurology. 124: 274-82. PMID 8287926 DOI: 10.1006/exnr.1993.1197  1
1993 Lester J, Fink S, Aronin N, DiFiglia M. Colocalization of D1 and D2 dopamine receptor mRNAs in striatal neurons. Brain Research. 621: 106-10. PMID 8221060 DOI: 10.1016/0006-8993(93)90303-5  1
1992 Huang Q, Zhou D, DiFiglia M. Neurobiotin, a useful neuroanatomical tracer for in vivo anterograde, retrograde and transneuronal tract-tracing and for in vitro labeling of neurons. Journal of Neuroscience Methods. 41: 31-43. PMID 1578900 DOI: 10.1016/0165-0270(92)90121-S  1
1992 Aronin N, DiFiglia M. The subcellular localization of the G-protein Gi alpha in the basal ganglia reveals its potential role in both signal transduction and vesicle trafficking. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 12: 3435-44. PMID 1527588  1
1992 Freese A, Finklestein SP, DiFiglia M. Basic fibroblast growth factor protects striatal neurons in vitro from NMDA-receptor mediated excitotoxicity. Brain Research. 575: 351-5. PMID 1349256 DOI: 10.1016/0006-8993(92)90104-H  1
1992 Huang Q, Vonsattel JP, Schaffer PA, Martuza RL, Breakefield XO, DiFiglia M. Introduction of a foreign gene (Escherichia coli lacZ) into rat neostriatal neurons using herpes simplex virus mutants: a light and electron microscopic study. Experimental Neurology. 115: 303-16. PMID 1311266 DOI: 10.1016/0014-4886(92)90196-W  1
1992 Huang Q, Zhou D, Chase K, Gusella JF, Aronin N, DiFiglia M. Immunohistochemical localization of the D1 dopamine receptor in rat brain reveals its axonal transport, pre- and postsynaptic localization, and prevalence in the basal ganglia, limbic system, and thalamic reticular nucleus. Proceedings of the National Academy of Sciences of the United States of America. 89: 11988-92. PMID 1281547  1
1991 Aronin N, Chase K, Sagar SM, Sharp FR, DiFiglia M. N-methyl-D-aspartate receptor activation in the neostriatum increases c-fos and fos-related antigens selectively in medium-sized neurons. Neuroscience. 44: 409-20. PMID 1834961 DOI: 10.1016/0306-4522(91)90065-V  1
1991 Aronin N, Chase K, Folsom R, Christakos S, DiFiglia M. Immunoreactive calcium-binding protein (calbindin-D28k) in interneurons and trigeminothalamic neurons of the rat nucleus caudalis localized with peroxidase and immunogold methods. Synapse (New York, N.Y.). 7: 106-13. PMID 1707189 DOI: 10.1002/syn.890070204  1
1990 Chiocca EA, Choi BB, Cai WZ, DeLuca NA, Schaffer PA, DiFiglia M, Breakefield XO, Martuza RL. Transfer and expression of the lacZ gene in rat brain neurons mediated by herpes simplex virus mutants. The New Biologist. 2: 739-46. PMID 2178004  1
1990 DiFiglia M, Roberts RC, Benowitz LI. Immunoreactive GAP-43 in the neuropil of adult rat neostriatum: localization in unmyelinated fibers, axon terminals, and dendritic spines. The Journal of Comparative Neurology. 302: 992-1001. PMID 2150524 DOI: 10.1002/cne.903020421  1
1990 Madras BK, Canfield DR, Pfaelzer C, Vittimberga FJ, Difiglia M, Aronin N, Bakthavachalam V, Baindur N, Neumeyer JL. Fluorescent and biotin probes for dopamine receptors: D1 and D2 receptor affinity and selectivity. Molecular Pharmacology. 37: 833-9. PMID 2141665  1
1990 Roberts RC, DiFiglia M. Evidence for synaptic proliferation, reorganization, and growth in the excitotoxic lesioned adult rat caudate nucleus. Experimental Neurology. 107: 1-10. PMID 2136832 DOI: 10.1016/0014-4886(90)90058-Z  1
1990 Roberts RC, DiFiglia M. Long-term survival of GABA-, enkephalin-, NADPH-diaphorase- and calbindin-d28k-containing neurons in fetal striatal grafts. Brain Research. 532: 151-9. PMID 1980852 DOI: 10.1016/0006-8993(90)91755-6  1
1990 Koroshetz WJ, Freese A, DiFiglia M. The correlation between excitatory amino acid-induced current responses and excitotoxicity in striatal cultures. Brain Research. 521: 265-72. PMID 1976414 DOI: 10.1016/0006-8993(90)91551-Q  1
1990 Freese A, DiFiglia M, Koroshetz WJ, Beal MF, Martin JB. Characterization and mechanism of glutamate neurotoxicity in primary striatal cultures. Brain Research. 521: 254-64. PMID 1976413 DOI: 10.1016/0006-8993(90)91550-Z  1
1990 DiFiglia M, Aronin N. Synaptic interactions between GABAergic neurons and trigeminothalamic cells in the rat trigeminal nucleus caudalis. Synapse (New York, N.Y.). 6: 358-63. PMID 1705057 DOI: 10.1002/syn.890060408  1
1990 DiFiglia M. Excitotoxic injury of the neostriatum: a model for Huntington's disease. Trends in Neurosciences. 13: 286-9. PMID 1695405 DOI: 10.1016/0166-2236(90)90111-M  1
1989 DiFiglia M, Christakos S, Aronin N. Ultrastructural localization of immunoreactive calbindin-D28k in the rat and monkey basal ganglia, including subcellular distribution with colloidal gold labeling. The Journal of Comparative Neurology. 279: 653-65. PMID 2918090 DOI: 10.1002/cne.902790411  1
1989 DiFiglia M, Marshall P, Covault J, Yamamoto M. Ultrastructural localization of molecular subtypes of immunoreactive neural cell adhesion molecule (NCAM) in the adult rodent striatum. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 9: 4158-68. PMID 2592994  1
1989 Roberts RC, DiFiglia M. Short- and long-term survival of large neurons in the excitotoxic lesioned rat caudate nucleus: a light and electron microscopic study. Synapse (New York, N.Y.). 3: 363-71. PMID 2525824 DOI: 10.1002/syn.890030410  1
1988 DiFiglia M, Schiff L, Deckel AW. Neuronal organization of fetal striatal grafts in kainate- and sham-lesioned rat caudate nucleus: light- and electron-microscopic observations. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 8: 1112-30. PMID 3357012  1
1988 Roberts RC, Difiglia M. Localization of immunoreactive GABA and enkephalin and NADPH-diaphorase-positive neurons in fetal striatal grafts in the quinolinic-acid-lesioned rat neostriatum. The Journal of Comparative Neurology. 274: 406-21. PMID 2975675 DOI: 10.1002/cne.902740309  1
1988 Difiglia M, Rafols JA. Synaptic organization of the globus pallidus. Journal of Electron Microscopy Technique. 10: 247-63. PMID 2906997 DOI: 10.1002/jemt.1060100304  1
1987 DiFiglia M. Synaptic organization of cholinergic neurons in the monkey neostriatum. The Journal of Comparative Neurology. 255: 245-58. PMID 3819015 DOI: 10.1002/cne.902550208  1
1987 Difiglia M, Graveland GA, Schiff L. Cytochrome oxidase activity in the rat caudate nucleus: light and electron microscopic observations. The Journal of Comparative Neurology. 255: 137-45. PMID 3029185 DOI: 10.1002/cne.902550111  1
1987 Rafols JA, Aronin N, Difiglia M. A Golgi study of the monkey paraventricular nucleus: neuronal types, afferent and efferent fibers. The Journal of Comparative Neurology. 257: 595-613. PMID 2447130 DOI: 10.1002/cne.902570408  1
1986 DiFiglia M, Carey J. Large neurons in the primate neostriatum examined with the combined Golgi-electron microscopic method. The Journal of Comparative Neurology. 244: 36-52. PMID 3950089 DOI: 10.1002/cne.902440104  1
1986 Aronin N, Chase K, DiFiglia M. Glutamic acid decarboxylase and enkephalin immunoreactive axon terminals in the rat neostriatum synapse with striatonigral neurons. Brain Research. 365: 151-8. PMID 3512036 DOI: 10.1016/0006-8993(86)90732-8  1
1985 Graveland GA, Williams RS, DiFiglia M. A Golgi study of the human neostriatum: neurons and afferent fibers. The Journal of Comparative Neurology. 234: 317-33. PMID 3988987 DOI: 10.1002/cne.902340304  1
1985 Graveland GA, DiFiglia M. The frequency and distribution of medium-sized neurons with indented nuclei in the primate and rodent neostriatum. Brain Research. 327: 307-11. PMID 3986508 DOI: 10.1016/0006-8993(85)91524-0  1
1985 Graveland GA, Williams RS, DiFiglia M. Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease. Science (New York, N.Y.). 227: 770-3. PMID 3155875  1
1984 Aronin N, Difiglia M, Graveland GA, Schwartz WJ, Wu JY. Localization of immunoreactive enkephalins in GABA synthesizing neurons of the rat neostriatum. Brain Research. 300: 376-80. PMID 6375810 DOI: 10.1016/0006-8993(84)90850-3  1
1984 Difiglia M, Aronin N, Leeman SE. Ultrastructural localization of immunoreactive neurotensin in the monkey superficial dorsal horn. The Journal of Comparative Neurology. 225: 1-12. PMID 6373845 DOI: 10.1002/cne.902250102  1
1984 DiFiglia M, Aronin N. Quantitative electron microscopic study of immunoreactive somatostatin axons in the rat neostriatum. Neuroscience Letters. 50: 325-31. PMID 6149504 DOI: 10.1016/0304-3940(84)90507-X  1
1984 DiFiglia M, Aronin N. Immunoreactive leu-enkephalin in the monkey hypothalamus including observations on its ultrastructural localization in the paraventricular nucleus. The Journal of Comparative Neurology. 225: 313-26. PMID 6144702 DOI: 10.1002/cne.902250302  1
1983 Maciewicz R, Phipps BS, Foote WE, Aronin N, DiFiglia M. The distribution of substance P-containing neurons in the cat Edinger-Westphal nucleus: relationship to efferent projection systems. Brain Research. 270: 217-30. PMID 6192874 DOI: 10.1016/0006-8993(83)90595-4  1
1982 Difiglia M, Pasik P, Pasik T. A Golgi and ultrastructural study of the monkey globus pallidus. The Journal of Comparative Neurology. 212: 53-75. PMID 7174908 DOI: 10.1002/cne.902120105  1
1982 DiFiglia M, Aronin N, Leeman SE. Light microscopic and ultrastructural localization of immunoreactive substance P in the dorsal horn of monkey spinal cord. Neuroscience. 7: 1127-39. PMID 6180349 DOI: 10.1016/0306-4522(82)91120-4  1
1982 DiFiglia M, Aronin N, Leeman SE. Immunoreactive substance P in the substantia nigra of the monkey: light and electron microscopic localization. Brain Research. 233: 381-8. PMID 6174178 DOI: 10.1016/0006-8993(82)91210-0  1
1982 DiFiglia M, Aronin N. Ultrastructural features of immunoreactive somatostatin neurons in the rat caudate nucleus. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 2: 1267-74. PMID 6126530  1
1982 DiFiglia M, Aronin N, Martin JB. Light and electron microscopic localization of immunoreactive Leu-enkephalin in the monkey basal ganglia. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 2: 303-20. PMID 6121017  1
1982 DiFiglia M, Aronin N, Leeman SE. Immunocytochemical study of neurotensin localization in the monkey spinal cord Annals of the New York Academy of Sciences. 405-408.  1
1981 Aronin N, DiFiglia M, Liotta AS, Martin JB. Ultrastructural localization and biochemical features of immunoreactive LEU-enkephalin in monkey dorsal horn. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 1: 561-77. PMID 6286896  1
1980 Difiglia M, Pasik P, Pasik T. Early postnatal development of the monkey neostriatum: a Golgi and ultrastructural study. The Journal of Comparative Neurology. 190: 303-31. PMID 6769982 DOI: 10.1002/cne.901900207  1
1980 Difiglia M, Pasik T, Pasik P. Ultrastructure of Golgi-impregnated and gold-toned spiny and aspiny neurons in the monkey neostriatum. Journal of Neurocytology. 9: 471-92. PMID 6160212 DOI: 10.1007/BF01204837  1
1979 DiFiglia M, Pasik T, Pasik P. Developmental aspects of neostriatal organization in monkeys. Applied Neurophysiology. 42: 81-3. PMID 110259  1
1978 Difiglia M, Pasik T, Pasik P. A Golgi study of afferent fibers in the neostriatum of monkeys. Brain Research. 152: 341-7. PMID 98215 DOI: 10.1016/0006-8993(78)90261-5  1
1976 Pasik P, Pasik T, DiFiglia M. Quantitative aspects of neuronal organization in the neostriatum of the macaque monkey. Research Publications - Association For Research in Nervous and Mental Disease. 55: 57-90. PMID 827000  1
1976 DiFiglia M, Pasik P, Pasik T. A Golgi study of neuronal types in the neostriatum of monkeys. Brain Research. 114: 245-56. PMID 822916 DOI: 10.1016/0006-8993(76)90669-7  1
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