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Papers authored by Difiglia M

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Mutant huntingtin and glycogen synthase kinase 3-beta accumulate in neuronal lipid rafts of a presymptomatic knock-in mouse model of Huntington's disease.

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Valencia A, Reeves PB, Sapp E, Li X, Alexander J, Kegel KB, Chase K, Aronin N, and DiFiglia M

Journal of neuroscience research 88(1):179-90, 2010 Jan - Who cited this? | PubMed ID: 19642201 | Fulltext

Single-step detection of mutant huntingtin in animal and human tissues: A bioassay for Huntington's disease.

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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, Zeitlin S,

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Analytical biochemistry 395(1):8-15, 2009 Dec 1 - Who cited this? | PubMed ID: 19664996 | Fulltext

Disruption of Rab11 activity in a knock-in mouse model of Huntington's disease.

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Li X, Sapp E, Chase K, Comer-Tierney LA, Masso N, Alexander J, Reeves P, Kegel KB, Valencia A, Esteves M, Aronin N, and Difiglia M

Neurobiology of disease 36(2):374-83, 2009 Nov - Who cited this? | PubMed ID: 19699304 | Fulltext

Mutant huntingtin impairs vesicle formation from recycling endosomes by interfering with Rab11 activity.

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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, Lifshitz L,

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Molecular and cellular biology 29(22):6106-16, 2009 Nov - Who cited this? | PubMed ID: 19752198 | Fulltext

Polyglutamine expansion in huntingtin increases its insertion into lipid bilayers.

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Kegel KB, Schewkunow V, Sapp E, Masso N, Wanker EE, DiFiglia M, and Goldmann WH

Biochemical and biophysical research communications 387(3):472-5, 2009 Sep 25 - Who cited this? | PubMed ID: 19607813 | Fulltext

Polyglutamine expansion in huntingtin alters its interaction with phospholipids.

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Kegel KB, Sapp E, Alexander J, Valencia A, Reeves P, Li X, Masso N, Sobin L, Aronin N, and DiFiglia M

Journal of neurochemistry 110(5):1585-97, 2009 Sep - Who cited this? | PubMed ID: 19566678 | Fulltext

Five siRNAs Targeting Three SNPs May Provide Therapy for Three-Quarters of Huntington's Disease Patients.

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Pfister EL, Kennington L, Straubhaar J, Wagh S, Liu W, Difiglia M, Landwehrmeyer B, Vonsattel JP, Zamore PD, and Aronin N

Current biology : CB 19(9):774-8, 2009 May 12 - Who cited this? | PubMed ID: 19361997 | Fulltext

A function of huntingtin in guanine nucleotide exchange on Rab11.

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Li X, Sapp E, Valencia A, Kegel KB, Qin ZH, Alexander J, Masso N, Reeves P, Ritch JJ, Zeitlin S, Aronin N, and Difiglia M

Neuroreport 19(16):1643-7, 2008 Oct 29 - Who cited this? | PubMed ID: 18845944 | Fulltext

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

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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, Blum JS,

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Autophagy 4(2):151 - Who cited this? | PubMed ID: 18188003 | Fulltext

Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits.

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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, and Aronin N

Proceedings of the National Academy of Sciences of the United States of America 104(43):17204, 2007 Oct 23 - Who cited this? | PubMed ID: 17940007 | Fulltext

Lysosomal proteases are involved in generation of N-terminal huntingtin fragments.

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Kim YJ, Sapp E, Cuiffo BG, Sobin L, Yoder J, Kegel KB, Qin ZH, Detloff P, Aronin N, and DiFiglia M

Neurobiology of disease 22(2):346-56, 2006 May - Who cited this? | PubMed ID: 16423528 | Fulltext

Susceptibility of striatal neurons to excitotoxic injury correlates with basal levels of Bcl-2 and the induction of P53 and c-Myc immunoreactivity.

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Liang ZQ, Wang XX, Wang Y, Chuang DM, DiFiglia M, Chase TN, and Qin ZH

Neurobiology of disease 20(2):562-73, 2005 Nov - Who cited this? | PubMed ID: 15922606 | Fulltext

Huntingtin associates with acidic phospholipids at the plasma membrane.

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Kegel KB, Sapp E, Yoder J, Cuiffo B, Sobin L, Kim YJ, Qin ZH, Hayden MR, Aronin N, Scott DL, Isenberg G, Goldmann WH, and DiFiglia M

The Journal of biological chemistry 280(43):36464-73, 2005 Oct 28 - Who cited this? | PubMed ID: 16085648 | Fulltext

Altered parvalbumin-positive neuron distribution in basal ganglia of individuals with Tourette syndrome.

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Kalanithi PS, Zheng W, Kataoka Y, DiFiglia M, Grantz H, Saper CB, Schwartz ML, Leckman JF, and Vaccarino FM

Proceedings of the National Academy of Sciences of the United States of America 102(37):13307-12, 2005 Sep 13 - Who cited this? | PubMed ID: 16131542 | Fulltext

Striatal potassium channel dysfunction in Huntington's disease transgenic mice.

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Ariano MA, Cepeda C, Calvert CR, Flores-Hernández J, Hernández-Echeagaray E, Klapstein GJ, Chandler SH, Aronin N, DiFiglia M, and Levine MS

Journal of neurophysiology 93(5):2565-74, 2005 May - Who cited this? | PubMed ID: 15625098 | Fulltext

Autophagy and its possible roles in nervous system diseases, damage and repair.

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Rubinsztein DC, DiFiglia M, Heintz N, Nixon RA, Qin ZH, Ravikumar B, Stefanis L, and Tolkovsky A

Autophagy 1(1):11-22, 2005 Apr - Who cited this? | PubMed ID: 16874045 | Fulltext

Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction.

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Qin ZH, Wang Y, Sapp E, Cuiffo B, Wanker E, Hayden MR, Kegel KB, Aronin N, and DiFiglia M

The Journal of neuroscience : the official journal of the Society for Neuroscience 24(1):269-81, 2004 Jan 7 - Who cited this? | PubMed ID: 14715959 | Fulltext

Autophagy regulates the processing of amino terminal huntingtin fragments.

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Qin ZH, Wang Y, Kegel KB, Kazantsev A, Apostol BL, Thompson LM, Yoder J, Aronin N, and DiFiglia M

Human molecular genetics 12(24):3231, 2003 Dec 15 - Who cited this? | PubMed ID: 14570716 | Fulltext

Distribution and ultrastructural localization of torsinA immunoreactivity in the human brain.

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Augood SJ, Keller-McGandy CE, Siriani A, Hewett J, Ramesh V, Sapp E, DiFiglia M, Breakefield XO, and Standaert DG

Brain research 986(1-2):12-21, 2003 Oct 3 - Who cited this? | PubMed ID: 12965225 | Fulltext

Huntingtin is degraded to small fragments by calpain after ischemic injury.

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Kim M, Roh JK, Yoon BW, Kang L, Kim YJ, Aronin N, and DiFiglia M

Experimental neurology 183(1):109-15, 2003 Sep - Who cited this? | PubMed ID: 12957494 | Fulltext

Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease.

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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, and Olson JM

Human molecular genetics 11(17):1939-51, 2002 Aug 15 - Who cited this? | PubMed ID: 12165556 | Fulltext

Huntingtin fragments that aggregate go their separate ways.

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DiFiglia M

Molecular cell 10(2):224-5, 2002 Aug - Who cited this? | PubMed ID: 12191468 | Fulltext

Striatal neurochemical changes in transgenic models of Huntington's disease.

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Ariano MA, Aronin N, Difiglia M, Tagle DA, Sibley DR, Leavitt BR, Hayden MR, and Levine MS

Journal of neuroscience research 68(6):716-29, 2002 Jun 15 - Who cited this? | PubMed ID: 12111832 | Fulltext

Maintenance of susceptibility to neurodegeneration following intrastriatal injections of quinolinic acid in a new transgenic mouse model of Huntington's disease.

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Petersén A, Chase K, Puschban Z, DiFiglia M, Brundin P, and Aronin N

Experimental neurology 175(1):297, 2002 May - Who cited this? | PubMed ID: 12009780 | Fulltext

Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription.

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Kegel KB, Meloni AR, Yi Y, Kim YJ, Doyle E, Cuiffo BG, Sapp E, Wang Y, Qin ZH, Chen JD, Nevins JR, Aronin N, and DiFiglia M

The Journal of biological chemistry 277(9):7466-76, 2002 Mar 1 - Who cited this? | PubMed ID: 11739372 | Fulltext

Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease.

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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, Levine MS,

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The Journal of neuroscience : the official journal of the Society for Neuroscience 21(23):9112-23, 2001 Dec 1 - Who cited this? | PubMed ID: 11717344 | Fulltext

Mice transgenic for exon 1 of the Huntington's disease gene display reduced striatal sensitivity to neurotoxicity induced by dopamine and 6-hydroxydopamine.

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Petersén A, Hansson O, Puschban Z, Sapp E, Romero N, Castilho RF, Sulzer D, Rice M, DiFiglia M, Przedborski S, and Brundin P

The European journal of neuroscience 14(9):1425-35, 2001 Nov - Who cited this? | PubMed ID: 11722604 | Fulltext

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.

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Kim YJ, Yi Y, Sapp E, Wang Y, Cuiffo B, Kegel KB, Qin ZH, Aronin N, and DiFiglia M

Proceedings of the National Academy of Sciences of the United States of America 98(22):12784-9, 2001 Oct 23 - Who cited this? | PubMed ID: 11675509 | Fulltext

Pro-caspase-8 is predominantly localized in mitochondria and released into cytoplasm upon apoptotic stimulation.

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Qin ZH, Wang Y, Kikly KK, Sapp E, Kegel KB, Aronin N, and DiFiglia M

The Journal of biological chemistry 276(11):8079-86, 2001 Mar 16 - Who cited this? | PubMed ID: 11102441 | Fulltext

Early and progressive accumulation of reactive microglia in the Huntington disease brain.

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Sapp E, Kegel KB, Aronin N, Hashikawa T, Uchiyama Y, Tohyama K, Bhide PG, Vonsattel JP, and DiFiglia M

Journal of neuropathology and experimental neurology 60(2):161-72, 2001 Feb - Who cited this? | PubMed ID: 11273004 | Fulltext

Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy.

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Kegel KB, Kim M, Sapp E, McIntyre C, Castaño JG, Aronin N, and DiFiglia M

The Journal of neuroscience : the official journal of the Society for Neuroscience 20(19):7268-78, 2000 Oct 1 - Who cited this? | PubMed ID: 11007884 | Fulltext

Genetics of childhood disorders: X. Huntington disease.

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DiFiglia M

Journal of the American Academy of Child and Adolescent Psychiatry 39(1):120-2, 2000 Jan - Who cited this? | PubMed ID: 10638076 | Fulltext

Are there multiple pathways in the pathogenesis of Huntington's disease?.

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Aronin N, Kim M, Laforet G, and DiFiglia M

Philosophical transactions of the Royal Society of London. Series B, Biological sciences 354(1386):995-1003, 1999 Jun 29 - Who cited this? | PubMed ID: 10434298 | Fulltext

Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition.

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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, and DiFiglia M

The Journal of neuroscience : the official journal of the Society for Neuroscience 19(3):964-73, 1999 Feb 1 - Who cited this? | PubMed ID: 9920660 | Fulltext

Axonal transport of N-terminal huntingtin suggests early pathology of corticostriatal projections in Huntington disease.

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Sapp E, Penney J, Young A, Aronin N, Vonsattel JP, and DiFiglia M

Journal of neuropathology and experimental neurology 58(2):165-73, 1999 Feb - Who cited this? | PubMed ID: 10029099 | Fulltext

Analysis of Huntingtin-associated protein 1 in mouse brain and immortalized striatal neurons.

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Martin EJ, Kim M, Velier J, Sapp E, Lee HS, Laforet G, Won L, Chase K, Bhide PG, Heller A, Aronin N, and Difiglia M

The Journal of comparative neurology 403(4):421-30, 1999 Jan 25 - Who cited this? | PubMed ID: 9888310 | Fulltext

Forskolin and dopamine D1 receptor activation increase huntingtin's association with endosomes in immortalized neuronal cells of striatal origin.

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Kim M, Velier J, Chase K, Laforet G, Kalchman MA, Hayden MR, Won L, Heller A, Aronin N, and Difiglia M

Neuroscience 89(4):1159-67, 1999 - Who cited this? | PubMed ID: 10362304 | Fulltext

Wild-type and mutant huntingtins function in vesicle trafficking in the secretory and endocytic pathways.

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Velier J, Kim M, Schwarz C, Kim TW, Sapp E, Chase K, Aronin N, and DiFiglia M

Experimental neurology 152(1):34-40, 1998 Jul - Who cited this? | PubMed ID: 9682010 | Fulltext

Huntington disease.

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Vonsattel JP and DiFiglia M

Journal of neuropathology and experimental neurology 57(5):369-84, 1998 May - Who cited this? | PubMed ID: 9596408 | Fulltext

Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions?.

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Davies SW, Beardsall K, Turmaine M, DiFiglia M, Aronin N, and Bates GP

Lancet 351(9096):131-3, 1998 Jan 10 - Who cited this? | PubMed ID: 9439509 | Fulltext

Huntingtin localization in brains of normal and Huntington's disease patients.

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Sapp E, Schwarz C, Chase K, Bhide PG, Young AB, Penney J, Vonsattel JP, Aronin N, and DiFiglia M

Annals of neurology 42(4):604-12, 1997 Oct - Who cited this? | PubMed ID: 9382472 | Fulltext

Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.

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DiFiglia M, Sapp E, Chase KO, Davies SW, Bates GP, Vonsattel JP, and Aronin N

Science (New York, N.Y.) 277(5334):1990-3, 1997 Sep 26 - Who cited this? | PubMed ID: 9302293 | Fulltext

Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation.

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Davies SW, Turmaine M, Cozens BA, DiFiglia M, Sharp AH, Ross CA, Scherzinger E, Wanker EE, Mangiarini L, and Bates GP

Cell 90(3):537-48, 1997 Aug 8 - Who cited this? | PubMed ID: 9267033 | Fulltext

Clinical Genetics, II. Huntington's disease: from the gene to pathophysiology.

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DiFiglia M

The American journal of psychiatry 154(8):1046, 1997 Aug - Who cited this? | PubMed ID: 9247386 | Fulltext

Fast transport and retrograde movement of huntingtin and HAP 1 in axons.

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Block-Galarza J, Chase KO, Sapp E, Vaughn KT, Vallee RB, DiFiglia M, and Aronin N

Neuroreport 8(9-10):2247-51, 1997 Jul 7 - Who cited this? | PubMed ID: 9243620 | Fulltext

Expression of normal and mutant huntingtin in the developing brain.

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Bhide PG, Day M, Sapp E, Schwarz C, Sheth A, Kim J, Young AB, Penney J, Golden J, Aronin N, and DiFiglia M

The Journal of neuroscience : the official journal of the Society for Neuroscience 16(17):5523-35, 1996 Sep 1 - Who cited this? | PubMed ID: 8757264 | Fulltext

Regional expression and subcellular localization of the tyrosine-specific phosphatase SH-PTP2 in the adult human nervous system.

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Reeves SA, Ueki K, Sinha B, Difiglia M, and Louis DN

Neuroscience 71(4):1037-42, 1996 Apr - Who cited this? | PubMed ID: 8684607 | Fulltext

Neuronal high-affinity glutamate transport in the rat central nervous system.

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Kanai Y, Bhide PG, DiFiglia M, and Hediger MA

Neuroreport 6(17):2357-62, 1995 Nov 27 - Who cited this? | PubMed ID: 8747153 | Fulltext

Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons.

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DiFiglia M, Sapp E, Chase K, Schwarz C, Meloni A, Young C, Martin E, Vonsattel JP, Carraway R, and Reeves SA

Neuron 14(5):1075-81, 1995 May - Who cited this? | PubMed ID: 7748555 | Fulltext

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.

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Huang Q, Zhou D, Sapp E, Aizawa H, Ge P, Bird ED, Vonsattel JP, and DiFiglia M

Neuroscience 65(2):397-407, 1995 Mar - Who cited this? | PubMed ID: 7777157 | Fulltext

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