Search Results - "Orr, HT"

Beyond the Qs in the polyglutamine diseases by Orr, H T

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RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia by Davidson, Beverly L, Xia, Haibin, Mao, Qinwen, Eliason, Steven L, Harper, Scott Q, Martins, Inês H, Orr, Harry T, Paulson, Henry L, Yang, Linda, Kotin, Robert M

“…The dominant polyglutamine expansion diseases, which include spinocerebellar ataxia type 1 (SCA1) and Huntington disease, are progressive, untreatable,…”
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Glutamine repeats and neurodegeneration by ZOGHBI, H. Y, ORR, H. T

“…A growing number of neurodegenerative diseases have been found to result from the expansion of an unstable trinucleotide repeat. Over the past 6 years,…”
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Recovery from Polyglutamine-Induced Neurodegeneration in Conditional SCA1 Transgenic Mice by Zu, Tao, Duvick, Lisa A, Kaytor, Michael D, Berlinger, Michael S, Zoghbi, Huda Y, Clark, H. Brent, Orr, Harry T

“…Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant, polyglutamine-induced neurodegenerative disorder that results in loss of motor coordination…”
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Interaction of Akt-Phosphorylated Ataxin-1 with 14-3-3 Mediates Neurodegeneration in Spinocerebellar Ataxia Type 1 by Chen, Hung-Kai, Fernandez-Funez, Pedro, Acevedo, Summer F., Lam, Yung C., Kaytor, Michael D., Fernandez, Michael H., Aitken, Alastair, Skoulakis, Efthimios M.C., Orr, Harry T., Botas, Juan, Zoghbi, Huda Y.

“…Spinocerebellar ataxia type 1 (SCA1) is one of several neurological disorders caused by a CAG repeat expansion. In SCA1, this expansion produces an abnormally…”
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The GSK3 β signaling cascade and neurodegenerative disease by Kaytor, Michael D, Orr, Harry T

“…Biochemical signaling pathways are known to have a critical role in neuronal development and function. A growing body of evidence is accumulating to suggest…”
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Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice by CUMMINGS, Christopher J, YALING SUN, OPAL, Puneet, ANTALFFY, Barbara, MESTRIL, Ruben, ORR, Harry T, DILLMANN, Wolfgang H, ZOGHBI, Huda Y

“…Many neurodegenerative diseases are caused by gain-of-function mechanisms in which the disease-causing protein is altered, becomes toxic to the cell, and…”
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The AXH Domain of Ataxin-1 Mediates Neurodegeneration through Its Interaction with Gfi-1/Senseless Proteins by Tsuda, Hiroshi, Jafar-Nejad, Hamed, Patel, Akash J., Sun, Yaling, Chen, Hung-Kai, Rose, Matthew F., Venken, Koen J.T., Botas, Juan, Orr, Harry T., Bellen, Hugo J., Zoghbi, Huda Y.

“…Spinocerebellar ataxia type 1 (SCA1) is a neurodegenerative disease caused by an expanded glutamine tract in human Ataxin-1 (hAtx-1). The expansion stabilizes…”
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Serine 776 of Ataxin-1 Is Critical for Polyglutamine-Induced Disease in SCA1 Transgenic Mice by Emamian, Effat S., Kaytor, Michael D., Duvick, Lisa A., Zu, Tao, Tousey, Susan K., Zoghbi, Huda Y., Clark, H.Brent, Orr, Harry T.

“…Polyglutamine-induced neurodegeneration in transgenic mice carrying the spinocerebellar ataxia type 1 (SCA1) gene is modulated by subcellular distribution of…”
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Neurodegenerative disease Neuron protection agency by Orr, Harry T

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Ataxin-1 Nuclear Localization and Aggregation: Role in Polyglutamine-Induced Disease in SCA1 Transgenic Mice by Klement, Ivan A, Skinner, Pamela J, Kaytor, Michael D, Yi, Hong, Hersch, Steven M, Clark, H.Brent, Zoghbi, Huda Y, Orr, Harry T

“…Transgenic mice carrying the spinocerebellar ataxia type 1 (SCA1) gene, a polyglutamine neurodegenerative disorder, develop ataxia with ataxin-1 localized to…”
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Gene profiling links SCA1 pathophysiology to glutamate signaling in Purkinje cells of transgenic mice by Serra, Heliane G., Byam, Courtney E., Lande, Jeffrey D., Tousey, Susan K., Zoghbi, Huda Y., Orr, Harry T.

“…Spinocerebellar ataxia type 1 (SCA1) is a neurodegenerative disease caused by the expansion of a polyglutamine repeat within the disease protein, ataxin 1. To…”
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Identification of genes that modify ataxin-1-induced neurodegeneration by Botas, Juan, Fernandez-Funez, Pedro, Nino-Rosales, Maria Laura, de Gouyon, Beatrice, She, Wei-Chi, Luchak, James M, Martinez, Pedro, Turiegano, Enrique, Benito, Jonathan, Capovilla, Maria, Skinner, Pamela J, McCall, Alanna, Canal, Inmaculada, Orr, Harry T, Zoghbi, Huda Y

“…A growing number of human neurodegenerative diseases result from the expansion of a glutamine repeat in the protein that causes the disease. Spinocerebellar…”
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Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1 by Zoghbi, Huda Y, Lin, Xi, Antalffy, Barbara, Kang, Dongcheul, Orr, Harry T

“…The expansion of an unstable CAG repeat causes spinocerebellar ataxia type 1 (SCA1) and several other neurodegenerative diseases. How polyglutamine expansions…”
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A Long CAG Repeat in the Mouse Sca1 Locus Replicates SCA1 Features and Reveals the Impact of Protein Solubility on Selective Neurodegeneration by Watase, Kei, Weeber, Edwin J., Xu, Bisong, Antalffy, Barbara, Yuva-Paylor, Lisa, Hashimoto, Kouichi, Kano, Masanobu, Atkinson, Richard, Sun, Yaling, Armstrong, Dawna L., Sweatt, J.David, Orr, Harry T., Paylor, Richard, Zoghbi, Huda Y.

“…To faithfully recreate the features of the human neurodegenerative disease spinocerebellar ataxia type 1 (SCA1) in the mouse, we targeted 154 CAG repeats into…”
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Mutation of the E6-AP Ubiquitin Ligase Reduces Nuclear Inclusion Frequency While Accelerating Polyglutamine-Induced Pathology in SCA1 Mice by Cummings, Christopher J, Reinstein, Eyal, Sun, Yaling, Antalffy, Barbara, Jiang, Yong-hui, Ciechanover, Aaron, Orr, Harry T, Beaudet, Arthur L, Zoghbi, Huda Y

“…Mutant ataxin-1, the expanded polyglutamine protein causing spinocerebellar ataxia type 1 (SCA1), aggregates in ubiquitin-positive nuclear inclusions (NI) that…”
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Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1 by Zoghbi, Huda Y, Cummings, Christopher J, Mancini, Michael A, Antalffy, Barbara, DeFranco, Donald B, Orr, Harry T

“…Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant neurodegenerative disorder caused by expansion of a polyglutamine tract in ataxin-1. In affected…”
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SUMOylation of the Polyglutamine Repeat Protein, Ataxin-1, Is Dependent on a Functional Nuclear Localization Signal by Riley, Brigit E., Zoghbi, Huda Y., Orr, Harry T.

“…SUMO (small ubiquitin-like modifier) is a member of the ubiquitin family of proteins. SUMO targets include proteins involved in numerous roles including…”
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RNA association and nucleocytoplasmic shuttling by ataxin-1 by Irwin, Stuart, Vandelft, Mark, Pinchev, Deborah, Howell, Jenny L, Graczyk, Joanna, Orr, Harry T, Truant, Ray

“…Spinocerebellar ataxia type 1 (SCA1) is a dominant neurodegenerative disease caused by the expression of mutant ataxin-1 containing an expanded polyglutamine…”
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Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures by Orr, Harry T, Skinner, Pamela J, Koshy, Beena T, Cummings, Christopher J, Klement, Ivan A, Helin, Kara, Servadio, Antonio, Zoghbi, Huda Y

“…Spinocerebellar ataxia type 1 (SCA1) is one of several neurodegenerative disorders caused by an expansion of a polyglutamine tract. It is characterized by…”
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