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...
CAG-encoded polyglutamine length polymorphism in the human genome (2007)
Butland, Stefanie L, Devon, Rebecca S, Huang, Yong, Mead, Carri-Lyn, Meynert, Alison M, Neal, Scott J, ...
Abstract Background Expansion of polyglutamine-encoding CAG trinucleotide repeats has been identified as the pathogenic mutation in nine different genes associated with neurodegenerative disorders....
Cocaine- and amphetamine-regulated transcript is increased in Huntington disease. (2007)
Björkqvist, Maria, Leavitt, Blair R, Nielsen, Jörgen E, Landwehrmeyer, Bernhard, Ecker, Daniel, Mulder, Hindrik, ...
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
Van Raamsdonk, Jeremy M, Pearson, Jacqueline, Murphy, Zoe, Hayden, Michael R, Leavitt, Blair R
Abstract Background Huntington disease (HD) is an adult onset neurodegenerative disorder caused by a polyglutamine expansion in the huntingtin (htt) protein. Htt function is essential for embryonic...
Mackenzie, Ian R, Butland, Stefanie L, Devon, Rebecca S, Dwosh, Emily, Feldman, Howard, Lindholm, Caroline, ...
Abstract Background Many cases of frontotemporal dementia (FTD) are familial, often with an autosomal dominant pattern of inheritance. Some are due to a mutation in the tau- encoding gene, on...
Body weight is modulated by levels of full-length Huntingtin (2006)
Van Raamsdonk, Jeremy M., Gibson, William T., Pearson, Jacqueline, Murphy, Zoe, Lu, Ge, Leavitt, Blair R., ...
Huntington disease is an adult-onset neurodegenerative disorder that is caused by the expansion of a polyglutamine tract within the Huntingtin (htt) protein. Wild-type htt has been shown to be...
Body weight is modulated by levels of full-length huntingtin (2006)
Van Raamsdonk, Jeremy M., Gibson, William T., Pearson, Jacqueline, Murphy, Zoe, Lu, Ge, Leavitt, Blair R., ...
Huntington disease (HD) is an adult onset neurodegenerative disorder that is caused by the expansion of a polyglutamine tract within the huntingtin (htt) protein. Wild-type htt has been shown to be...
Missirlis, Perseus I, Mead, Carri-Lyn R, Butland, Stefanie L, Ouellette, BF Francis, Devon, Rebecca S, Leavitt, Blair R, ...
Abstract Background To date, 35 human diseases, some of which also exhibit anticipation, have been associated with unstable repeats. Anticipation has been reported in a number of diseases in which...
Van Raamsdonk, Jeremy M., Pearson, Jacqueline, Rogers, Daniel A., Bissada, Nagat, Vogl, A. Wayne, Hayden, Michael R., ...
Huntington disease (HD) is an adult-onset neurodegenerative disease caused by a toxic gain of function in the huntingtin (htt) protein. The contribution of wild-type htt function to the pathogenesis...
Van Raamsdonk, Jeremy M., Murphy, Zoe, Slow, Elizabeth J., Leavitt, Blair R., Hayden, Michael R.
Huntington disease (HD) is an adult onset neurodegenerative disorder that predominantly affects the striatum and cortex despite ubiquitous expression of mutant huntingtin (htt). Here we demonstrate...
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease (2003)
Slow, Elizabeth J., Van Raamsdonk, Jeremy, Rogers, Daniel, Coleman, Sarah H., Graham, Rona K., Deng, Yu, ...
An expanded CAG repeat is the underlying genetic defect in Huntington disease, a disorder characterized by motor, psychiatric and cognitive deficits and striatal atrophy associated with neuronal...
Zeron, Melinda M, Hansson, Oskar, Chen, Nansheng, Wellington, Cheryl L, Leavitt, Blair R, Brundin, Patrik, ...
Previous work suggests N-methyl-D-aspartate receptor (NMDAR) activation may be involved in degeneration of medium-sized spiny striatal neurons in Huntington's disease (HD). Here we show that these...
Chan, Edmond Y.W., Luthi-Carter, Ruth, Strand, Andrew, Solano, Steven M., Hanson, Sarah A., DeJohn, Molly M., ...
Both transcriptional dysregulation and proteolysis of mutant huntingtin (htt) are postulated to be important components of Huntington's disease (HD) pathogenesis. In previous studies, we demonstrated...
Singaraja, Roshni R., Hadano, Shinji, Metzler, Martina, Givan, Scott, Wellington, Cheryl L., Warby, Simon, ...
Huntington disease (HD) is caused by polyglutamine [poly(Q)] expansion in the protein huntingtin (htt). Although the exact mechanism of disease progression remains to be elucidated, altered...
Missirlis, Perseus I, Mead, Carri-Lyn R, Butland, Stefanie L, Ouellette, BF Francis, Devon, Rebecca S, Leavitt, Blair R, ...
Slow, Elizabeth J., Graham, Rona K., Osmand, Alexander P., Devon, Rebecca S., Lu, Ge, Deng, Yu, ...
We have serendipitously established a mouse that expresses an N-terminal human huntingtin (htt) fragment with an expanded polyglutamine repeat (≈120) under the control of the endogenous human...
Wild-Type Huntingtin Reduces the Cellular Toxicity of Mutant Huntingtin In Vivo
Leavitt, Blair R., Guttman, Julian A., Hodgson, J. Graeme, Kimel, Gil H., Singaraja, Roshni, Vogl, A. Wayne, ...
We have developed yeast artificial chromosome (YAC) transgenic mice expressing normal (YAC18) and mutant (YAC46 or YAC72) human huntingtin (htt), in a developmental- and tissue-specific manner, that...
Mackenzie, Ian R, Butland, Stefanie L, Devon, Rebecca S, Dwosh, Emily, Feldman, Howard, Lindholm, Caroline, ...
Missirlis, Perseus I, Mead, Carri-Lyn R, Butland, Stefanie L, Ouellette, BF Francis, Devon, Rebecca S, Leavitt, Blair R, ...
Slow, Elizabeth J., Graham, Rona K., Osmand, Alexander P., Devon, Rebecca S., Lu, Ge, Deng, Yu, ...
We have serendipitously established a mouse that expresses an N-terminal human huntingtin (htt) fragment with an expanded polyglutamine repeat (≈120) under the control of the endogenous human...
Wild-Type Huntingtin Reduces the Cellular Toxicity of Mutant Huntingtin In Vivo
Leavitt, Blair R., Guttman, Julian A., Hodgson, J. Graeme, Kimel, Gil H., Singaraja, Roshni, Vogl, A. Wayne, ...
We have developed yeast artificial chromosome (YAC) transgenic mice expressing normal (YAC18) and mutant (YAC46 or YAC72) human huntingtin (htt), in a developmental- and tissue-specific manner, that...
Mackenzie, Ian R, Butland, Stefanie L, Devon, Rebecca S, Dwosh, Emily, Feldman, Howard, Lindholm, Caroline, ...
CAG-encoded polyglutamine length polymorphism in the human genome
Butland, Stefanie L, Devon, Rebecca S, Huang, Yong, Mead, Carri-Lyn, Meynert, Alison M, Neal, Scott J, ...
Huntingtin inhibits caspase-3 activation
Zhang, Yu, Leavitt, Blair R, Van Raamsdonk, Jeremy M, Dragatsis, Ioannis, Goldowitz, Dan, MacDonald, Marcy E, ...
Huntington's disease results from a mutation in the HD gene encoding for the protein huntingtin. The function of huntingtin, although beginning to be elucidated, remains largely unclear. To probe the...
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...
Brain-specific Proteins Decline in the Cerebrospinal Fluid of Humans with Huntington Disease*S⃞
Fang, Qiaojun, Strand, Andrew, Law, Wendy, Faca, Vitor M., Fitzgibbon, Matthew P., Hamel, Nathalie, ...
We integrated five sets of proteomics data profiling the constituents of cerebrospinal fluid (CSF) derived from Huntington disease (HD)-affected and -unaffected individuals with genomics data...