Search Results - "Difiglia, M."

Therapeutic Silencing of Mutant Huntingtin with siRNA Attenuates Striatal and Cortical Neuropathology and Behavioral Deficits by DiFiglia, M., Sena-Esteves, M., Chase, K., Sapp, E., Pfister, E., Sass, M., Yoder, J., Reeves, P., Pandey, R. K., Rajeev, K. G., Manoharan, M., Sah, D. W. Y., Zamore, P. D., Aronin, N.

“…Huntington's disease (HD) is a neurodegenerative disorder caused by expansion of a CAG repeat in the huntingtin (Htt) gene. HD is autosomal dominant and, in…”
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Early and Progressive Accumulation of Reactive Microglia in the Huntington Disease Brain by SAPP, E, KEGEL, K B, ARONIN, N, HASHIKAWA, T, UCHIYAMA, Y, TOHYAMA, K, BHIDE, P G, VONSATTEL, J P, DIFIGLIA, M

“…Microglia may contribute to cell death in neurodegenerative diseases. We studied the activation of microglia in affected regions of Huntington disease (HD)…”
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Huntingtin Expression Stimulates Endosomal-Lysosomal Activity, Endosome Tubulation, and Autophagy by Kegel, Kimberly B, Kim, Manho, Sapp, Ellen, McIntyre, Charmian, Castano, Jose G, Aronin, Neil, DiFiglia, Marian

“…An expansion of polyglutamines in the N terminus of huntingtin causes Huntington's disease (HD) and results in the accrual of mutant protein in the nucleus and…”
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Huntingtin localization in brains of normal and Huntington's disease patients by Sapp, E, Schwarz, C, Chase, K, Bhide, P G, Young, A B, Penney, J, Vonsattel, J P, Aronin, N, DiFiglia, M

“…The immunohistochemical localization of huntingtin was examined in the Huntington's disease (HD) brain with an antibody that recognizes the wild-type and…”
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Changes in Cortical and Striatal Neurons Predict Behavioral and Electrophysiological Abnormalities in a Transgenic Murine Model of Huntington's Disease by Laforet, Genevieve A, Sapp, Ellen, Chase, Kathryn, McIntyre, Charmian, Boyce, Frederick M, Campbell, Mary, Cadigan, Beth A, Warzecki, Lori, Tagle, Danilo A, Reddy, P. Hemachandra, Cepeda, Carlos, Calvert, Christopher R, Jokel, Eve S, Klapstein, Gloria J, Ariano, Marjorie A, Levine, Michael S, DiFiglia, Marian, Aronin, Neil

“…Neurons in Huntington's disease exhibit selective morphological and subcellular alterations in the striatum and cortex. The link between these neuronal changes…”
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Maintenance of Susceptibility to Neurodegeneration Following Intrastriatal Injections of Quinolinic Acid in a New Transgenic Mouse Model of Huntington's Disease by Petersén, Å., Chase, K., Puschban, Z., DiFiglia, M., Brundin, P., Aronin, N.

“…A transgenic mouse model of Huntington's disease (R6/1 and R6/2 lines) expressing exon 1 of the HD gene with 115-150 CAG repeats resisted striatal damage…”
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Clinical Genetics, II. Huntington's disease: from the gene to pathophysiology by DiFiglia, M

“…Huntington's disease, an autosomal dominant neurodegenerative brain disorder manifested by progressively deteriorating motor, cognitive and mood symptoms, is…”
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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 by Huang, Q, Zhou, D, Chase, K, Gusella, J F, Aronin, N, DiFiglia, M

“…D1 dopamine receptor localization was examined by immunohistochemistry using a polyclonal anti-peptide antibody which (i) immunoprecipitated a protein fragment…”
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Evidence for a preferential loss of enkephalin immunoreactivity in the external globus pallidus in low grade Huntington's disease using high resolution image analysis by Sapp, E., Ge, P., Aizawa, H., Bird, E., Penney, J., Young, A.B., Vonsattel, J.-P., Difiglia, M.

“…Previous studies have shown that in advanced cases of Huntington's disease, enkephalin-immunoreactive striatal projections to the external globus pallidus may…”
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Evidence for Degenerative and Regenerative Changes in Neostriatal Spiny Neurons in Huntington's Disease by Graveland, G. A., Williams, R. S., DiFiglia, M.

“…Golgi impregnations of neostriatum from deceased Huntington's disease patients and controls were examined. In all cases of Huntington's disease the morphology…”
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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 by Huang, Q, Zhou, D, Sapp, E, Aizawa, H, Ge, P, Bird, E D, Vonsattel, J P, DiFiglia, M

“…In Huntington's disease striatal neurons undergo marked changes in dendritic morphology and coincidently exhibit an increase in immunoreactive calbindin D28k…”
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Forskolin and dopamine D1 receptor activation increase huntingtin's association with endosomes in immortalized neuronal cells of striatal origin by KIM, M, VELIER, J, CHASE, K, LAFORET, G, KALCHMAN, M. A, HAYDEN, M. R, WON, L, HELLER, A, ARONIN, N, DIFIGLIA, M

“…Huntingtin is a cytoplasmic protein of unknown function that associates with vesicle membranes and microtubules. Its protein interactions suggest that…”
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The frequency and distribution of medium-sized neurons with indented nuclei in the primate and rodent neostriatum by Graveland, G A, DiFiglia, M

“…Nuclear morphology is known to distinguish two classes of medium-sized neurons in the neostriatum, spiny projection neurons which have an unindented nucleus…”
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Quinolinic acid-induced increases in calbindin D 28k immunoreactivity in rat striatal neurons in vivo and in vitro mimic the pattern seen in Huntington's disease by Huang, Q., Zhou, D., Sapp, E., Aizawa, H., Ge, P., Bird, E.D., Vonsattel, J.-P., DiFiglia, M.

“…In Huntington's disease striatal neurons undergo marked changes in dendritic morphology and coincidently exhibit an increase in immunoreactive calbindin D 28k…”
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Ultrastructural features of immunoreactive somatostatin neurons in the rat caudate nucleus by DiFiglia, M, Aronin, N

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Ultrastructural localization of molecular subtypes of immunoreactive neural cell adhesion molecule (NCAM) in the adult rodent striatum by DiFiglia, M, Marshall, P, Covault, J, Yamamoto, M

“…Neural cell adhesion molecule (NCAM) is a plasma membrane glycoprotein that is thought to mediate adhesion between neuronal elements and play an important role…”
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Altered Parvalbumin-Positive Neuron Distribution in Basal Ganglia of Individuals with Tourette Syndrome by Paul S. A. Kalanithi, Zheng, Wei, Kataoka, Yuko, DiFiglia, Marian, Grantz, Heidi, Saper, Clifford B., Schwartz, Michael L., Leckman, James F., Vaccarino, Flora M., Llinas, Rodolfo R.

“…Tourette syndrome (TS) is a childhood neuropsychiatric disorder characterized by motor and vocal tics. Imaging studies found alterations in caudate (Cd) and…”
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A Golgi and ultrastructural study of the monkey globus pallidus by Difiglia, M, Pasik, P, Pasik, T

“…Golgi preparations reveal that the most frequent type of pallidal neuron (principal cell), which has been recognized in all previous reports, is large (20-50…”
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N-methyl-D-aspartate receptor activation in the neostriatum increases c-fos and fos-related antigens selectively in medium-sized neurons by Aronin, N, Chase, K, Sagar, S M, Sharp, F R, DiFiglia, M

“…In the neostriatum a selective loss of neurons occurs following exposure to N-methyl-D-aspartate receptor agonists. One hypothesis emerging from this…”
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Huntington Disease by G. Vonsattel, Jean Paul, DiFiglia, Marian

“…Huntington disease (HD) is an autosomal dominant neurodegenerative disorder with midlife onset characterized by psychiatric, cognitive, and motor symptoms…”
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