Benn, Caroline L, Fox, Helen, Bates, Gillian P
Abstract Background Transcriptional dysregulation is an early, key pathogenic mechanism in Huntington's disease (HD). Therefore, gene expression analyses have biomarker potential for measuring...
Kuhn, Alexandre, Goldstein, Darlene R., Hodges, Angela, Strand, Andrew D., Sengstag, Thierry, Kooperberg, Charles, ...
To test the hypotheses that mutant huntingtin protein length and wild-type huntingtin dosage have important effects on disease-related transcriptional dysfunction, we compared the changes in mRNA in...
Zourlidou, Alexandra, Gidalevitz, Tali, Kristiansen, Mark, Landles, Christian, Woodman, Ben, Wells, Dominic J., ...
Huntington's disease (HD) is caused by an expanded polyglutamine tract in the huntingtin protein. Mitochondrial dysfunction and free radical damage occur in both R6/2 mice and HD patient brains and...
Increased metabolism in the R6/2 mouse model of Huntington's disease. (2007)
Bacos, Karl, Wood, Nigel I, Lindqvist, Andreas, Wierup, Nils, Woodman, Ben, ...
Abstract is not available
Re Kuhn, Darlene R. Goldstein, Angela Hodges, Andrew D. Str, Thierry Sengstag, Charles Kooperberg, ...
Mutant huntingtin's effects on striatal gene expression in mice
Zourlidou, Alexandra, Gidalevitz, Tali, Kristiansen, Mark, Landles, Christian, Woodman, Ben, Wells, Dominic J., ...
Huntington's disease (HD) is caused by an expanded polyglutamine tract in the huntingtin protein. Mitochondrial dysfunction and free radical damage occur in both R6/2 mice and HD patient brains and...
Bett, John S., Goellner, Geoffrey M., Woodman, Ben, Pratt, Gregory, Rechsteiner, Martin, Bates, Gillian P.
Huntington's disease (HD) is one of a group of neurodegenerative disorders caused by the pathological expansion of a glutamine tract. A hallmark of these so-called polyglutamine diseases is the...
Bett, John S., Goellner, Geoffrey M., Woodman, Ben, Pratt, Gregory, Rechsteiner, Martin, Bates, Gillian P.
Björkqvist, Maria, Petersén, Åsa, Bacos, Karl, Isaacs, Jeremy, Norlén, Per, Gil, Joana, ...
Huntington's disease (HD) is characterised by a triad of motor, psychiatric, and cognitive symptoms. While many of these symptoms are likely to be related to central nervous system pathology, others...
Björkqvist, Maria, Petersén, Åsa, Bacos, Karl, Isaacs, Jeremy, Norlén, Per, Gil, Joana, ...
Huntington's disease (HD) is characterized by a triad of motor, psychiatric and cognitive symptoms. Although many of these symptoms are likely to be related to central nervous system pathology,...
Smith, Ruben, Petersén, Asa, Bates, Gillian P, Brundin, Patrik, Li, Jia-Yi
Abstract is not available
Reduction of GnRH and infertility in the R6/2 mouse model of Huntington's disease. (2005)
Papalexi, Eugenia, Persson, Anna, Bjorkqvist, Maria, Petersén, Asa, Woodman, Ben, Bates, Gillian P, ...
Abstract is not available
Benn, Caroline L., Landles, Christian, Li, He, Strand, Andrew D., Woodman, Ben, Sathasivam, Kirupa, ...
In postmortem Huntington's disease brains, mutant htt is present in both nuclear and cytoplasmic compartments. To dissect the impact of nuclear and extranuclear mutant htt on the initiation and...
Hay, David G., Sathasivam, Kirupa, Tobaben, Sönke, Stahl, Bernd, Marber, Michael, Mestril, Ruben, ...
The manipulation of chaperone levels has been shown to inhibit aggregation and/or rescue cell death in cell culture, S. cerevisiae, C. elegans and D. melanogaster models of Huntington's disease (HD)...
Hay, David G., Sathasivam, Kirupa, Tobaben, Sönke, Stahl, Bernd, Marber, Michael, Mestril, Ruben, ...
The manipulation of chaperone levels has been shown to inhibit aggregation and/or rescue cell death in Saccharomyces cerevisiae, Caenorhabditis elegans, Drosophila melanogaster and cell culture...
Hay, David G., Sathasivam, Kirupa, Tobaben, Sönke, Stahl, Bernd, Marber, Michael, Mestril, Ruben, ...
The manipulation of chaperone levels has been shown to inhibit aggregation and/or rescue cell death in cell culture, S. cerevisiae, C. elegans and D. melanogaster models of Huntington's disease (HD)...
Mouse models of Huntington's disease (2002)
Radically updated to include coverage of developments in the molecular biology and genetics of the condition following isolation of the gene. Includes implications for prediction and genetic...
Waelter, Stephanie, Scherzinger, Eberhard, Hasenbank, Renate, Nordhoff, Eckhard, Lurz, Rudi, Goehler, Heike, ...
The huntingtin interacting protein (HIP1) is enriched in membrane-containing cell fractions and has been implicated in vesicle trafficking. It is a multidomain protein containing an N-terminal ENTH...
Sathasivam, Kirupa, Woodman, Ben, Mahal, Amabirpal, Bertaux, Fabien, Wanker, Erich E., Shima, Dave T., ...
Huntington’s disease (HD) is a progressive neurological disorder caused by a CAG/polyglutamine repeat expansion. We have previously generated the R6/2 mouse model that expresses exon 1 of...
Li, He, Li, Shi-Hua, Cheng, Anna L., Mangiarini, Laura, Bates, Gillian P., Li, Xiao-Jiang
How aggregates of polyglutamine proteins are involved in the neurological symptoms of glutamine repeat diseases is unknown. We show that huntingtin aggregates are present in the neuronal processes of...
Hadano, Shinji, Ishida, Yoshikazu, Tomiyasu, Hitoshi, Yamamoto, Kenji, Bates, Gillian P., Ikeda, Joh-E
Six hundred and twenty-seven cDNA clones from human brain cDNA libraries were characterized and integrated into a transcript map of the 1-Mb region on human chromosome 4p16.3 containing the...
Tommerup, Niels, Aagaard, Lissi, Lund, Connie L., Boel, Esper, Baxendale, Sarah, Bates, Gillian P., ...
Chromosomal aneusomy is a major cause of reproductive wastage and congenital malformations in man. Zinc finger encoding genes would be good candidates for being involved in the multiple developmental...
Scherzinger, Eberhard, Sittler, Annie, Schweiger, Katja, Heiser, Volker, Lurz, Rudi, Hasenbank, Renate, ...
Huntington’s disease is a progressive neurodegenerative disorder caused by a polyglutamine [poly(Q)] repeat expansion in the first exon of the huntingtin protein. Previously, we showed that...
Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease
Turmaine, Mark, Raza, Aysha, Mahal, Amarbirpal, Mangiarini, Laura, Bates, Gillian P., Davies, Stephen W.
Huntington's disease (HD) is a fatal inherited neurodegenerative disorder characterized by personality changes, motor impairment, and subcortical dementia. HD is one of a number of diseases caused by...
Steffan, Joan S., Kazantsev, Aleksey, Spasic-Boskovic, Olivera, Greenwald, Marilee, Zhu, Ya-Zhen, Gohler, Heike, ...
Huntington's Disease (HD) is caused by an expansion of a polyglutamine tract within the huntingtin (htt) protein. Pathogenesis in HD appears to include the cytoplasmic cleavage of htt and release of...
Cha, Jang-Ho J., Kosinski, Christoph M., Kerner, Julie A., Alsdorf, Stephen A., Mangiarini, Laura, Davies, Stephen W., ...
Loss of neurotransmitter receptors, especially glutamate and dopamine receptors, is one of the pathologic hallmarks of brains of patients with Huntington disease (HD). Transgenic mice that express...
Hockly, Emma, Richon, Victoria M., Woodman, Benjamin, Smith, Donna L., Zhou, Xianbo, Rosa, Eddie, ...
Huntington's disease (HD) is an inherited, progressive neurological disorder that is caused by a CAG/polyglutamine repeat expansion and for which there is no effective therapy. Recent evidence...
Zhang, Xiaoqian, Smith, Donna L., Meriin, Anatoli B., Engemann, Sabine, Russel, Deborah E., Roark, Margo, ...
Polyglutamine (polyQ) disorders, including Huntington's disease (HD), are caused by expansion of polyQ-encoding repeats within otherwise unrelated gene products. In polyQ diseases, the pathology and...
Huntingtin and the molecular pathogenesis of Huntington's disease
Landles, Christian, Bates, Gillian P.
Fourth in Molecular Medicine Review Series
Scherzinger, Eberhard, Sittler, Annie, Schweiger, Katja, Heiser, Volker, Lurz, Rudi, Hasenbank, Renate, ...
Huntington’s disease is a progressive neurodegenerative disorder caused by a polyglutamine [poly(Q)] repeat expansion in the first exon of the huntingtin protein. Previously, we showed that...
Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease
Turmaine, Mark, Raza, Aysha, Mahal, Amarbirpal, Mangiarini, Laura, Bates, Gillian P., Davies, Stephen W.
Huntington's disease (HD) is a fatal inherited neurodegenerative disorder characterized by personality changes, motor impairment, and subcortical dementia. HD is one of a number of diseases caused by...
Steffan, Joan S., Kazantsev, Aleksey, Spasic-Boskovic, Olivera, Greenwald, Marilee, Zhu, Ya-Zhen, Gohler, Heike, ...
Huntington's Disease (HD) is caused by an expansion of a polyglutamine tract within the huntingtin (htt) protein. Pathogenesis in HD appears to include the cytoplasmic cleavage of htt and release of...
Cha, Jang-Ho J., Kosinski, Christoph M., Kerner, Julie A., Alsdorf, Stephen A., Mangiarini, Laura, Davies, Stephen W., ...
Loss of neurotransmitter receptors, especially glutamate and dopamine receptors, is one of the pathologic hallmarks of brains of patients with Huntington disease (HD). Transgenic mice that express...
Hockly, Emma, Richon, Victoria M., Woodman, Benjamin, Smith, Donna L., Zhou, Xianbo, Rosa, Eddie, ...
Huntington's disease (HD) is an inherited, progressive neurological disorder that is caused by a CAG/polyglutamine repeat expansion and for which there is no effective therapy. Recent evidence...
Zhang, Xiaoqian, Smith, Donna L., Meriin, Anatoli B., Engemann, Sabine, Russel, Deborah E., Roark, Margo, ...
Polyglutamine (polyQ) disorders, including Huntington's disease (HD), are caused by expansion of polyQ-encoding repeats within otherwise unrelated gene products. In polyQ diseases, the pathology and...
Huntingtin and the molecular pathogenesis of Huntington's disease
Landles, Christian, Bates, Gillian P.
Fourth in Molecular Medicine Review Series
DNA instability in postmitotic neurons
Gonitel, Roman, Moffitt, Hilary, Sathasivam, Kirupa, Woodman, Ben, Detloff, Peter J., Faull, Richard L. M., ...
Huntington's disease (HD) is caused by a CAG repeat expansion that is unstable upon germ-line transmission and exhibits mosaicism in somatic tissues. We show that region-specific CAG repeat mosaicism...
Björkqvist, Maria, Wild, Edward J., Thiele, Jenny, Silvestroni, Aurelio, Andre, Ralph, Lahiri, Nayana, ...
Huntington's disease (HD) is an inherited neurodegenerative disorder characterized by both neurological and systemic abnormalities. We examined the peripheral immune system and found widespread...
Bett, John S., Cook, Casey, Petrucelli, Leonard, Bates, Gillian P.
Impairment of the ubiquitin-proteasome system (UPS) has long been considered an attractive hypothesis to explain the selective dysfunction and death of neurons in polyglutamine disorders such as...
Benn, Caroline L., Butler, Rachel, Mariner, Lydia, Nixon, Jude, Moffitt, Hilary, Mielcarek, Michal, ...
Huntington's disease (HD) is an inherited, progressive neurological disorder caused by a CAG/polyglutamine repeat expansion, for which there is no effective disease modifying therapy. In recent...