A Broad Phenotypic Screen Identifies Novel Phenotypes Driven by a Single Mutant Allele in Huntington’s Disease CAG Knock-In Mice
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Edith T. Lopez | H. Fuchs | T. Gillis | M. MacDonald | J. Seidman | E. Wolf | H. Schulz | H. Wakimoto | M. D. de Angelis | S. Hölter | V. Gailus-Durner | M. Klingenspor | V. Wheeler | J. Rozman | T. Klopstock | S. Cotman | L. Becker | W. Hans | B. Rathkolb | L. Garrett | Jong-Min Lee | L. Glasl | M. Kovalenko | Jolene R. Guide | M. Stromberg | Alexander A. Götz | Anja Schrewed | Wolfgang Wursta | Lisa Glasl
[1] R. Gold,et al. Hepatic mitochondrial dysfunction in manifest and premanifest Huntington disease , 2013, Neurology.
[2] Mei Kwan,et al. Comprehensive Behavioral and Molecular Characterization of a New Knock-In Mouse Model of Huntington’s Disease: zQ175 , 2012, PloS one.
[3] A. Hannan,et al. Neurocardiac dysregulation and neurogenic arrhythmias in a transgenic mouse model of Huntington's disease , 2012, The Journal of physiology.
[4] Tongbin Li,et al. Homeostatic Adaptations in Brain Energy Metabolism in Mouse Models of Huntington Disease , 2012, Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.
[5] S. Dunnett,et al. Light and electron microscopic characterization of the evolution of cellular pathology in HdhQ92 Huntington's disease knock-in mice , 2012, Brain Research Bulletin.
[6] S. Dunnett,et al. Longitudinal analysis of the behavioural phenotype in Hdh (CAG)150 Huntington's disease knock-in mice , 2012, Brain Research Bulletin.
[7] S. Dunnett,et al. Light and electron microscopic characterization of the evolution of cellular pathology in the Hdh(CAG)150 Huntington's disease knock-in mouse , 2012, Brain Research Bulletin.
[8] S. Dunnett,et al. Early onset deficits on the delayed alternation task in the HdhQ92 knock-in mouse model of Huntington's disease , 2012, Brain Research Bulletin.
[9] S. Dunnett,et al. Five choice serial reaction time performance in the HdhQ92 mouse model of Huntington's disease , 2012, Brain Research Bulletin.
[10] S. Dunnett,et al. Longitudinal analysis of the behavioural phenotype in HdhQ92 Huntington's disease knock-in mice , 2012, Brain Research Bulletin.
[11] Richard L. Doty,et al. Olfactory dysfunction in Parkinson disease , 2012, Nature Reviews Neurology.
[12] N. Torquet,et al. Activation of adult-born neurons facilitates learning and memory , 2012, Nature Neuroscience.
[13] A. Giralt,et al. Long-term memory deficits in Huntington's disease are associated with reduced CBP histone acetylase activity. , 2012, Human molecular genetics.
[14] Jane S. Paulsen,et al. CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion , 2012, Neurology.
[15] F. Saudou,et al. Huntington's disease knock-in male mice show specific anxiety-like behaviour and altered neuronal maturation , 2012, Neuroscience Letters.
[16] M. Štrucl,et al. Microcirculation response to local cooling in patients with Huntington’s disease , 2012, Journal of Neurology.
[17] R. Schiffmann,et al. Early Alterations of Brain Cellular Energy Homeostasis in Huntington Disease Models* , 2011, The Journal of Biological Chemistry.
[18] C. Colwell,et al. Baroreceptor reflex dysfunction in the BACHD mouse model of Huntington’s disease. , 2011, PLoS currents.
[19] E. Cattaneo,et al. Emerging roles for cholesterol in Huntington's disease , 2011, Trends in Neurosciences.
[20] T. Gillis,et al. Quantification of Age-Dependent Somatic CAG Repeat Instability in Hdh CAG Knock-In Mice Reveals Different Expansion Dynamics in Striatum and Liver , 2011, PloS one.
[21] M. MacDonald,et al. Differential effects of the Huntington's disease CAG mutation in striatum and cerebellum are quantitative not qualitative. , 2011, Human molecular genetics.
[22] B. Thorens. Brain glucose sensing and neural regulation of insulin and glucagon secretion , 2011, Diabetes, obesity & metabolism.
[23] M. MacDonald,et al. HD CAG-correlated gene expression changes support a simple dominant gain of function. , 2011, Human molecular genetics.
[24] L. Lanfumey,et al. Sexually Dimorphic Serotonergic Dysfunction in a Mouse Model of Huntington's Disease and Depression , 2011, PloS one.
[25] Steve D. M. Brown,et al. High-throughput mouse phenotyping. , 2011, Methods.
[26] R. Mandel,et al. Longitudinal behavioral, cross-sectional transcriptional and histopathological characterization of a knock-in mouse model of Huntington's disease with 140 CAG repeats , 2011, Experimental Neurology.
[27] R. Barker,et al. Body composition in premanifest Huntington’s disease reveals lower bone density compared to controls. , 2011, PLoS currents.
[28] B. Christie,et al. Altered adult hippocampal neurogenesis in the YAC128 transgenic mouse model of Huntington disease , 2011, Neurobiology of Disease.
[29] Donald A. Wilson,et al. Sniffing out the contributions of the olfactory tubercle to the sense of smell: Hedonics, sensory integration, and more? , 2011, Neuroscience & Biobehavioral Reviews.
[30] H. Fuchs,et al. Mouse phenotyping. , 2011, Methods.
[31] N. Secher,et al. Reduced gluconeogenesis and lactate clearance in Huntington's disease , 2010, Neurobiology of Disease.
[32] T. Dick,et al. Modulation of cardiorespiratory function mediated by the paraventricular nucleus , 2010, Respiratory Physiology & Neurobiology.
[33] R. Albin,et al. Early alterations of autophagy in Huntington disease-like mice , 2010, Autophagy.
[34] Julia Noack,et al. Different importance of the volatile and non-volatile fractions of an olfactory signature for individual social recognition in rats versus mice and short-term versus long-term memory , 2010, Neurobiology of Learning and Memory.
[35] J. Marinus,et al. Autonomic symptoms in patients and pre‐manifest mutation carriers of Huntington’s disease , 2010, European journal of neurology.
[36] N. Wood,et al. Disruption of Peripheral Circadian Timekeeping in a Mouse Model of Huntington's Disease and Its Restoration by Temporally Scheduled Feeding , 2010, The Journal of Neuroscience.
[37] S. Cregan,et al. Metabotropic Glutamate Receptor-Mediated Cell Signaling Pathways Are Altered in a Mouse Model of Huntington's Disease , 2010, The Journal of Neuroscience.
[38] B. Peterlin,et al. Autonomic dysfunction in presymptomatic and early symptomatic Huntington’s disease , 2009, Acta neurologica Scandinavica.
[39] M. MacDonald,et al. Huntingtin facilitates polycomb repressive complex 2 , 2009, Human molecular genetics.
[40] W. Brooks,et al. Reduced limbic and hypothalamic volumes correlate with bone density in early Alzheimer's disease. , 2010, Journal of Alzheimer's disease : JAD.
[41] M. Mehler,et al. Impairment of developmental stem cell-mediated striatal neurogenesis and pluripotency genes in a knock-in model of Huntington's disease , 2009, Proceedings of the National Academy of Sciences.
[42] G. Bates,et al. Formation of Polyglutamine Inclusions in a Wide Range of Non-CNS Tissues in the HdhQ150 Knock-In Mouse Model of Huntington's Disease , 2009, PloS one.
[43] S. Dunnett,et al. Rule learning, visuospatial function and motor performance in the HdhQ92 knock-in mouse model of Huntington's disease , 2009, Behavioural Brain Research.
[44] T. Hansen,et al. Huntington’s Disease Does Not Appear to Increase the Risk of Diabetes Mellitus , 2009, Journal of neuroendocrinology.
[45] M. MacDonald,et al. Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models , 2009, Neurobiology of Disease.
[46] J. Willey,et al. PRESTROKE PHYSICAL ACTIVITY IS ASSOCIATED WITH SEVERITY AND LONG-TERM OUTCOME FROM FIRST-EVER STROKE , 2009, Neurology.
[47] P. Brundin,et al. Beyond the brain: widespread pathology in Huntington's disease , 2009, The Lancet Neurology.
[48] M. MacDonald,et al. Rrs1 Is Involved in Endoplasmic Reticulum Stress Response in Huntington Disease* , 2009, The Journal of Biological Chemistry.
[49] Edith T. Lopez,et al. Intergenerational and striatal CAG repeat instability in Huntington's disease knock-in mice involve different DNA repair genes , 2009, Neurobiology of Disease.
[50] Matthew T. Lorincz,et al. Expanded CAG repeats in the murine Huntington's disease gene increases neuronal differentiation of embryonic and neural stem cells , 2009, Molecular and Cellular Neuroscience.
[51] W. Brooks,et al. Bone density and brain atrophy in early Alzheimer's disease. , 2009, Journal of Alzheimer's disease : JAD.
[52] H. Fuchs,et al. Systemic first-line phenotyping. , 2009, Methods in molecular biology.
[53] Richard Axel,et al. Mice with a “Monoclonal Nose”: Perturbations in an Olfactory Map Impair Odor Discrimination , 2008, Neuron.
[54] M. Chesselet,et al. Extensive early motor and non-motor behavioral deficits are followed by striatal neuronal loss in knock-in Huntington's disease mice , 2008, Neuroscience.
[55] T. Stijnen,et al. Weight loss in Huntington disease increases with higher CAG repeat number , 2008, Neurology.
[56] R. Albin,et al. Rodent genetic models of Huntington disease , 2008, Neurobiology of Disease.
[57] J. Cordes,et al. Cardiovagal modulation upon postural change is altered in Huntington’s disease , 2008, European journal of neurology.
[58] S. Dunnett,et al. Time course of choice reaction time deficits in the HdhQ92 knock-in mouse model of Huntington's disease in the operant Serial Implicit Learning Task (SILT) , 2008, Behavioural Brain Research.
[59] V. Kostic,et al. Glucose homeostasis in Huntington disease: abnormalities in insulin sensitivity and early-phase insulin secretion. , 2008, Archives of neurology.
[60] T. Shimazaki,et al. [Mammalian neural stem cells]. , 2008, Tanpakushitsu kakusan koso. Protein, nucleic acid, enzyme.
[61] A. Nørremølle,et al. Impaired Glucose Tolerance in the R6/1 Transgenic Mouse Model of Huntington’s Disease , 2007, Journal of neuroendocrinology.
[62] Jane S. Paulsen,et al. Detection of Huntington’s disease decades before diagnosis: the Predict-HD study , 2007, Journal of Neurology, Neurosurgery, and Psychiatry.
[63] Roger L Albin,et al. Longitudinal Evaluation of the Hdh (CAG)150 Knock-In Murine Model of Huntington's Disease , 2007, The Journal of Neuroscience.
[64] C. Blakemore,et al. Olfactory abnormalities in Huntington’s disease: Decreased plasticity in the primary olfactory cortex of R6/1 transgenic mice and reduced olfactory discrimination in patients , 2007, Brain Research.
[65] Danielle A. Simmons,et al. Brain-Derived Neurotrophic Factor Restores Synaptic Plasticity in a Knock-In Mouse Model of Huntington's Disease , 2007, The Journal of Neuroscience.
[66] G. Rebec,et al. Sex differences in behavior and striatal ascorbate release in the 140 CAG knock-in mouse model of Huntington's disease , 2007, Behavioural Brain Research.
[67] K. Hoyt,et al. Cardiac dysfunction in the R6/2 mouse model of Huntington’s disease , 2007, Neurobiology of Disease.
[68] Juan Fernandez-Ruiz,et al. Olfaction and neurodegeneration in HD , 2007, Neuroreport.
[69] S. Dunnett,et al. The operant serial implicit learning task reveals early onset motor learning deficits in the HdhQ92 knock‐in mouse model of Huntington's disease , 2007, The European journal of neuroscience.
[70] N. Aziz,et al. Hypothalamic Dysfunction and Neuroendocrine and Metabolic Alterations in Huntington Disease: Clinical Consequences and Therapeutic Implications , 2007, Reviews in the neurosciences.
[71] F. Richer,et al. Episodic memory impairment in Huntington's disease: A meta-analysis , 2006, Neuropsychologia.
[72] M. Larsson,et al. Olfactory Functions in Asymptomatic Carriers of the Huntington Disease Mutation , 2006, Journal of clinical and experimental neuropsychology.
[73] Samuel Garcia,et al. Neurogenic correlates of an olfactory discrimination task in the adult olfactory bulb , 2006, The European journal of neuroscience.
[74] Ronald Wetzel,et al. Behavioral abnormalities precede neuropathological markers in rats transgenic for Huntington's disease. , 2006, Human molecular genetics.
[75] D. Devanand,et al. Olfactory dysfunction as a predictor of neurodegenerative disease , 2006, Current neurology and neuroscience reports.
[76] Edith T. Lopez,et al. Genetic background modifies nuclear mutant huntingtin accumulation and HD CAG repeat instability in Huntington's disease knock-in mice. , 2006, Human molecular genetics.
[77] S. Tabrizi,et al. Progressive alterations in the hypothalamic-pituitary-adrenal axis in the R6/2 transgenic mouse model of Huntington's disease. , 2006, Human molecular genetics.
[78] S. Dunnett,et al. Selective extra-dimensional set shifting deficit in a knock-in mouse model of Huntington's disease , 2006, Brain Research Bulletin.
[79] C. Blakemore,et al. Neurogenesis in the R6/1 transgenic mouse model of Huntington's disease: effects of environmental enrichment , 2006, The European journal of neuroscience.
[80] J. Lucas,et al. Reduced expression of the TrkB receptor in Huntington's disease mouse models and in human brain , 2006, The European journal of neuroscience.
[81] P. Brundin,et al. Reduced hippocampal neurogenesis in R6/2 transgenic Huntington's disease mice , 2005, Neurobiology of Disease.
[82] O. Gavrilova,et al. Increased glucose tolerance and reduced adiposity in the absence of fasting hypoglycemia in mice with liver-specific Gs alpha deficiency. , 2005, The Journal of clinical investigation.
[83] M. MacDonald,et al. HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism. , 2005, Human molecular genetics.
[84] B Fischl,et al. Regional cortical thinning in preclinical Huntington disease and its relationship to cognition , 2005, Neurology.
[85] Werner Müller,et al. Introducing the German Mouse Clinic: open access platform for standardized phenotyping , 2005, Nature Methods.
[86] B. Peterlin,et al. Early sympathetic hyperactivity in Huntington's disease , 2004, European journal of neurology.
[87] E. Vandenbussche,et al. Cognitive changes in patients with Huntington’s disease (HD) and asymptomatic carriers of the HD mutation , 2004, Journal of Neurology.
[88] C. Blakemore,et al. Decreased hippocampal cell proliferation in R6/1 Huntington's mice , 2004, Neuroreport.
[89] J R Hodges,et al. Profile of cognitive progression in early Huntington’s disease , 2003, Neurology.
[90] M. Chesselet,et al. Time course of early motor and neuropathological anomalies in a knock‐in mouse model of Huntington's disease with 140 CAG repeats , 2003, The Journal of comparative neurology.
[91] M. MacDonald,et al. Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice. , 2003, Human molecular genetics.
[92] J. Mysore,et al. Identification of a presymptomatic molecular phenotype in Hdh CAG knock-in mice. , 2002, Human molecular genetics.
[93] H. Przuntek,et al. Autonomic nervous system function in Huntington's disease , 2002, Journal of neurology, neurosurgery, and psychiatry.
[94] M. MacDonald,et al. Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice. , 2002, Human molecular genetics.
[95] R. Albin,et al. Neurological abnormalities in a knock-in mouse model of Huntington's disease. , 2001, Human molecular genetics.
[96] B. Gulyás,et al. Olfactory Functions Are Mediated by Parallel and Hierarchical Processing , 2000, Neuron.
[97] M. MacDonald,et al. Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice. , 2000, Human molecular genetics.
[98] J. Vincent,et al. Importance of newly generated neurons in the adult olfactory bulb for odor discrimination. , 2000, Proceedings of the National Academy of Sciences of the United States of America.
[99] Jane S. Paulsen,et al. Odor detection, learning, and memory in Huntington's disease , 1999, Journal of the International Neuropsychological Society.
[100] J. Romano,et al. Sympathetic skin response and heart rate variability in patients with Huntington disease. , 1999, Archives of neurology.
[101] Harper Ps. Huntington's disease: a clinical, genetic and molecular model for polyglutamine repeat disorders. , 1999 .
[102] S. W. Davies,et al. Formation of polyglutamine inclusions in non-CNS tissue. , 1999, Human molecular genetics.
[103] H. Johnston,et al. A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice. , 1999, Human molecular genetics.
[104] M. Hurlbert,et al. Mice transgenic for an expanded CAG repeat in the Huntington's disease gene develop diabetes. , 1999, Diabetes.
[105] P. Harper. Huntington's disease: a clinical, genetic and molecular model for polyglutamine repeat disorders. , 1999, Philosophical transactions of the Royal Society of London. Series B, Biological sciences.
[106] Jacqueline K. White,et al. Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse. , 1999, Human molecular genetics.
[107] S. File,et al. Striking changes in anxiety in Huntington's disease transgenic mice , 1998, Brain Research.
[108] Jacqueline K. White,et al. Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion , 1997, Nature Genetics.
[109] P. Brennan,et al. NEURAL MECHANISMS OF MAMMALIAN OLFACTORY LEARNING , 1997, Progress in Neurobiology.
[110] P. Moberg,et al. Odor identification in Huntington's disease patients and asymptomatic gene carriers. , 1997, The Journal of neuropsychiatry and clinical neurosciences.
[111] T. Robbins,et al. Executive and mnemonic functions in early Huntington's disease. , 1996, Brain : a journal of neurology.
[112] E. Benarroch. The central autonomic network: functional organization, dysfunction, and perspective. , 1993, Mayo Clinic proceedings.
[113] Manish S. Shah,et al. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes , 1993, Cell.
[114] D. Lanska,et al. Huntington's disease mortality in the United States , 1988, Neurology.
[115] L. Farrer. Diabetes mellitus in Huntington disease , 1985, Clinical genetics.
[116] E. Chiu,et al. Causes of death in Huntington's Disease , 1982, The Medical journal of Australia.
[117] S. Podolsky,et al. Abnormal glucose tolerance and arginine tolerance tests in Huntington's disease. , 1977, Gerontology.
[118] Shirley Hansen,et al. Huntington's Chorea , 1973 .
[119] J. Bolt,et al. Huntington's chorea. A study of liver function and histology. , 1973, The Quarterly journal of medicine.