Search Results - "Sharp, Alan H."

A Mutant Ataxin-3 Putative-Cleavage Fragment in Brains of Machado-Joseph Disease Patients and Transgenic Mice Is Cytotoxic above a Critical Concentration by Goti, Daniel, Katzen, Scott M, Mez, Jesse, Kurtis, Noam, Kiluk, Jennifer, Ben-Haiem, Lea, Jenkins, Nancy A, Copeland, Neal G, Kakizuka, Akira, Sharp, Alan H, Ross, Christopher A, Mouton, Peter R, Colomer, Veronica

“…Machado-Joseph disease (MJD) is an inherited neurodegenerative disorder caused by ataxin-3 with a polyglutamine expansion. It is proposed that a toxic cleavage…”
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Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation by Davies, S W, Turmaine, M, Cozens, B A, DiFiglia, M, Sharp, A H, Ross, C A, Scherzinger, E, Wanker, E E, Mangiarini, L, Bates, G P

“…Huntington's disease (HD) is one of an increasing number of human neurodegenerative disorders caused by a CAG/polyglutamine-repeat expansion. The mutation…”
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Lymphocyte Apoptosis: Mediation by Increased Type 3 Inositol 1,4,5- Trisphosphate Receptor by Khan, Adil A., Soloski, Mark J., Sharp, Alan H., Schilling, Gabriele, Sabatini, David M., Li, Shi-Hua, Ross, Christopher A., Snyder, Solomon H.

“…B and T lymphocytes undergoing apoptosis in response to anti-immunoglobulin M antibodies and dexamethasone, respectively, were found to have increased amounts…”
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Intranuclear Inclusions and Neuritic Aggregates in Transgenic Mice Expressing a Mutant N-Terminal Fragment of Huntingtin by Schilling, Gabriele, Becher, Mark W., Sharp, Alan H., Jinnah, Hyder A., Duan, Kui, Kotzuk, Joyce A., Slunt, Hilda H., Ratovitski, Tamara, Cooper, Jillian K., Jenkins, Nancy A., Copeland, Neal G., Price, Donald L., Ross, Christopher A., Borchelt, David R.

“…Huntington's disease (HD) is an inherited, neurodegenerative disorder caused by the expansion of a glutamine repeat in the N-terminus of the huntingtin…”
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Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization by Snyder, Solomon H, Sawa, Akira, Wiegand, Gordon W, Cooper, Jillian, Margolis, Russell L, Sharp, Alan H, Lawler, Joseph F, Greenamyre, J. Timothy, Ross, Christopher A

“…Huntington disease (HD) is a genetically dominant condition caused by expanded CAG repeats coding for glutamine in the HD gene product huntingtin. Although HD…”
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Localization of inositol trisphosphate receptor subtype 3 to insulin and somatostatin secretory granules and regulation of expression in islets and insulinoma cells by Blondel, O, Moody, M M, Depaoli, A M, Sharp, A H, Ross, C A, Swift, H, Bell, G I

“…Calcium ions play a central role in stimulus-secretion coupling in pancreatic beta cells, and an elevation of cytosolic Ca(2+) levels is necessary for insulin…”
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Synphilin-1 associates with α-synuclein and promotes the formation of cytosolic inclusions by Sharp, Alan H, Troncoso, Juan C, Amaravi, Ravi K, Guo, Xin, Kleiderlein, John J, Engelender, Simone, Worley, Paul F, Kaminsky, Zachary, Lanahan, Anthony A, Dawson, Valina L, Margolis, Russell L, Ross, Christopher A, Dawson, Ted M

“…Parkinson disease (PD) is a neurodegenerative disease characterized by tremor, bradykinesia, rigidity and postural instability. Post-mortem examination shows…”
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A huntingtin-associated protein enriched in brain with implications for pathology by Li, Xiao-Jiang, Li, Shi-Hua, Sharp, Alan H, Nucifora, Frederick C, Schilling, Gabriele, Lanahan, Anthony, Worley, Paul, Snyder, Solomon H, Ross, Christopher A

“…Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by an expanding polyglutamine repeat in the IT15 or huntingtin gene…”
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Widespread expression of Huntington's disease gene (IT15) protein product by Sharp, Alan H, Loev, Scott J, Schilling, Gabriele, Li, Shi-Hua, Li, Xiao-Jiang, Bao, Jun, Wagster, Molly V, Kotzuk, Joyce A, Steiner, Joseph P, Lo, Amy, Hedreen, John, Sisodia, Sangram, Snyder, Solomon H, Dawson, Ted M, Ryugo, David K, Ross, Christopher A

“…Huntington's Disease (HD) is caused by expansion of a CAG repeat within a putative open reading frame of a recently identified gene, IT15. We have examined the…”
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Differential cellular expression of isoforms of inositol 1,4,5-triphosphate receptors in neurons and glia in brain by Sharp, Alan H., Nucifora Jr, Frederick C., Blondel, Olivier, Sheppard, Carol A., Zhang, Chuanyi, Snyder, Solomon H., Russell, James T., Ryugoand, David K., Ross, Christopher A.

“…Inositol 1,4,5‐trisphosphate receptors (IP3R) are mediators of second messenger‐induced intracellular calcium release. Three isoforms are known to be expressed…”
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Huntingtin's WW domain partners in Huntington's disease post-mortem brain fulfill genetic criteria for direct involvement in Huntington's disease pathogenesis by PASSANI, L. A, BEDFORD, M. T, FABER, P. W, MCGINNIS, K. M, SHARP, A. H, GUSELLA, J. F, VONSATTEL, J.-P, MACDONALD, M. E

“…An elongated glutamine tract in mutant huntingtin initiates Huntington's disease (HD) pathogenesis via a novel structural property that displays neuronal…”
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Neurobiology of Huntington's Disease by Sharp, Alan H., Ross, Christopher A.

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Cleavage of Atrophin-1 at Caspase Site Aspartic Acid 109 Modulates Cytotoxicity by Ellerby, L M, Andrusiak, R L, Wellington, C L, Hackam, A S, Propp, S S, Wood, J D, Sharp, A H, Margolis, R L, Ross, C A, Salvesen, G S, Hayden, M R, Bredesen, D E

“…Dentatorubropallidoluysian atrophy (DRPLA) is one of eight autosomal dominant neurodegenerative disorders characterized by an abnormal CAG repeat expansion…”
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Polyglutamine pathogenesis by Ross, Christopher A., Wood, Jonathan D., Schilling, Gabriele, Peters, Matthew F., Nucifora, Frederick C., Cooper, Jillian K., Sharp, Alan H., Margolis, Russell L., Borchelt, David R.

“…An increasing number of neurodegenerative disorders have been found to be caused by expanding CAG triplet repeats that code for polyglutamine. Huntington's…”
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Expansion of Polyglutamine Repeat in Huntingtin Leads to Abnormal Protein Interactions Involving Calmodulin by Bao, Jun, Sharp, Alan H., Wagster, Molly V., Becher, Mark, Schilling, Gabriele, Ross, Christopher A., Dawson, Valina L., Dawson, Ted M.

“…Huntington's disease (HD) is an inherited neurodegenerative disorder associated with expansion of a CAG repeat in the IT15 gene. The IT15 gene is translated to…”
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Huntingtin-Associated Protein (HAP1): Discrete Neuronal Localizations in the Brain Resemble those of Neuronal Nitric Oxide Synthase by Li, Xiao-Jiang, Sharp, Alan H., Li, Shi-Hua, Dawson, Ted M., Snyder, Solomon H., Ross, Christopher A.

“…Huntington disease stems from a mutation of the protein huntingtin and is characterized by selective loss of discrete neuronal populations in the brain…”
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Sequence and expression of MRNAs encoding the α1 and α2 subunits of a DHP-sensitive calcium channel by ELLIS, S. B, WILLIAMS, M. E, SCHWARTZ, A, HARPOL, M. M, WAYS, N. R, BRENNER, R, SHARP, A. H, LEUNG, A. T, CAMPBELL, K. P, MCKENNA, E, KOCH, W. J, HUI, A

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The biochemistry and molecular biology of the dihydropyridine-sensitive calcium channel by Campbell, K P, Leung, A T, Sharp, A H

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Expression of the Huntington's disease (IT15) protein product in HD patients by Schilling, G, Sharp, A H, Loev, S J, Wagster, M V, Li, S H, Stine, O C, Ross, C A

“…Huntington's disease (HD) is an inherited, neurodegenerative disorder caused by expansion of a CAG repeat in the IT15 gene, leading to an expanded glutamine…”
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HAP1-huntingtin interactions do not contribute to the molecular pathology in Huntington's disease transgenic mice by Bertaux, Fabien, Sharp, Alan H, Ross, Christopher A, Lehrach, Hans, Bates, Gillian P, Wanker, Erich

“…HAP1 (huntingtin associated protein) has previously been found to interact with huntingtin (htt) in a glutamine length dependent manner and has been proposed…”
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