Gene expression and behaviour in mouse models of HD

Brain Research Bulletin

Volumn 88, Issue 2-3, 2012, Pages 276-284

  Bowles, K R ^a   Brooks, S P ^b   Dunnett, S B ^b   Jones, L ^a  

^a CARDIFF UNIVERSITY   (United Kingdom)

^b CARDIFF UNIVERSITY   (United Kingdom)

Author keywords

Behaviour; Epigenetics; Gene expression; Huntington's disease; Mouse models; Transcription

Indexed keywords

BIOLOGICAL MARKER; HUNTINGTIN;

ANIMAL BEHAVIOR; BRAIN ATROPHY; DISEASE COURSE; DISEASE MODEL; EXON; GENE EXPRESSION; GENE INDUCTION; GENE INSERTION; GENE TARGETING; HUNTINGTON CHOREA; MOUSE; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; REVIEW; TRANSGENIC MOUSE;

ANIMALS; BEHAVIOR, ANIMAL; DISEASE MODELS, ANIMAL; GENE EXPRESSION REGULATION; HUMANS; HUNTINGTON DISEASE; MICE; MICE, TRANSGENIC; SEROTONIN PLASMA MEMBRANE TRANSPORT PROTEINS;

EID: 84860833787     PISSN: 03619230     EISSN: 18732747     Source Type: Journal    
DOI: 10.1016/j.brainresbull.2011.07.021     Document Type: Review

References (135)

1. Alarcon J.M., et al. Chromatin acetylation, memory, and LTP are impaired in CBP+/- mice: a model for the cognitive deficit in Rubinstein-Taybi syndrome and its amelioration. Neuron 2004, 42(6):947-959.
2. Arzberger T., et al. Changes of NMDA receptor subunit (NR1, NR2B) and glutamate transporter (GLT1) mRNA expression in Huntington's disease-an in situ hybridization study. Journal of Neuropathology and Experimental Neurology 1997, 56(4):440-454.
3. Augood S.J., et al. Reduction in enkephalin and substance P messenger RNA in the striatum of early grade Huntington's disease: a detailed cellular in situ hybridization study. Neuroscience 1996, 72(4):1023-1036.
4. Augood S.J., Faull R.L.M., Emson P.C. Dopamine D-1 and D-2 receptor gene expression in the striatum in Huntington's disease. Annals of Neurology 1997, 42(2):215-221.
5. Bae B.I., et al. p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease. Neuron 2005, 47(1):29-41.
6. Bayram-Weston Z., et al. Light and electron microscopic characterization of the evolution of cellular pathology in HdhQ92 Huntington's disease knock-in mice. Brain Research Bulletin 2012, 88:189-198.
7. Bayram-Weston Z., et al. Light and electron microscopic characterization of the evolution of cellular pathology in the R6/1. Huntington's disease transgenic mice. Brain Research Bulletin 2012, 88:104-112.
8. Bayram-Weston Z., et al. Light and electron microscopic characterization of the evolution of cellular pathology in YAC128 transgenic mice. Brain Research Bulletin 2012, 88:137-147.
9. Bayram-Weston Z., et al. Light and electron microscopic characterization of the evolution of cellular pathology in the Hdh(CAG)150 Huntington's disease knock-in mouse. Brain Research Bulletin 2012, 88:189-198.
10. Bender A., et al. Creatine improves health and survival of mice. Neurobiology of Aging 2008, 29(9):1404-1411.
11. Benn C.L., et al. Environmental enrichment reduces neuronal intranuclear inclusion load but has no effect on messenger RNA expression in a mouse model of Huntington disease. Journal of Neuropathology and Experimental Neurology 2010, 69(8):817-827.
12. Bitton D.A., et al. Exon level integration of proteomics and microarray data. BMC Bioinformatics 2008, 9:11.
13. Bordner K.A., et al. Parallel declines in cognition, motivation, and locomotion in aging mice: Association with immune gene upregulation in the medial prefrontal cortex. Experimental Gerontology 2011, 46:643-659.
14. Borovecki F., et al. Genome-wide expression profiling of human blood reveals biomarkers for Huntington's disease. Proceedings of the National Academy of Sciences of the United States of America 2005, 102(31):11023-11028.
15. Boutell J.M., et al. Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin. Human Molecular Genetics 1999, 8(9):1647-1655.
16. Brooks S.P., et al. Longitudinal analysis of the behavioural phenotype in R6/1 (C57BL/6J) Huntington's disease transgenic mice. Brain Research Bulletin 2012, 88:94-103.
17. Brooks S., et al. Longitudinal analysis of the behavioural phenotype in YAC128 (C57BL/6J) Huntington's disease transgenic mice. Brain Research Bulletin 2012, 88:113-120.
18. Brooks S., et al. Longitudinal analysis of the behavioural phenotype in HdhQ92 Huntington's disease knock-in mice. Brain Research Bulletin 2012, 88:148-155.
19. Brooks S., et al. Longitudinal analysis of the behavioural phenotype in Hdh(CAG)150 Huntington's disease knock-in mice. Brain Research Bulletin 2012, 88:182-188.
20. Buckley N.J., et al. The role of REST in transcriptional and epigenetic dysregulation in Huntington's disease. Neurobiology of disease 2010, 39(1):28-39.
21. Buschow S.I., et al. Dominant processes during human dendritic cell maturation revealed by integration of proteome and transcriptome at the pathway level. Journal of Proteome Research 2010, 9(4):1727-1737.
22. Cha J.H.J. Transcriptional signatures in Huntington's disease. Progress in Neurobiology 2007, 83(4):228-248.
23. Chan E.Y.W., et al. Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease. Human Molecular Genetics 2002, 11(17):1939-1951.
24. Crook Z.R., Housman D. Huntington's disease can mice lead the way to treatment?. Neuron 2011, 69:423-435.
25. Cui L.B., et al. Transcriptional repression of PGC-alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. Cell 2006, 127(1):59-69.
26. D'Amours G., et al. Differential gene expression profiling in the mouse brain during motor skill learning: focus on the striatum structure. Behavioural Brain Research 2011, 221(1):108-117.
27. Davies S.W., et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 1997, 90:537-548.
28. Deschepper M., et al. Proteomic changes in the brains of Huntington's disease mouse models reflect pathology and implicate mitochondrial changes. Brain Research Bulletin 2012, 88:210-222.
29. Desplats P.A., et al. Selective deficits in the expression of striatal-enriched mRNAs in Huntington's disease. Journal of Neurochemistry 2006, 96(3):743-757.
30. Djoussé L., et al. Weight loss in early stage of Huntington's disease. Neurology 2002, 1325-1330.
31. Dowie M.J., et al. Altered CB1 receptor and endocannabinoid levels precede motor symptom onset in a transgenic mouse model of huntington's disease. Neuroscience 2009, 163(1):456-465.
32. Dulac C. Brain function and chromatin plasticity. Nature 2010, 465(7299):728-735.
33. Dunah A.W., et al. Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease. Science 2002, 296(5576):2238-2243.
34. El Rawas R., et al. Early exposure to environmental enrichment alters the expression of genes of the endocannabinoid system. Brain Research 2011, 1390:80-89.
35. Ferrante R.J. Mouse models of Huntington's disease and methodological considerations for therapeutic trials. Biochimica et Biophysica Acta 2009, 1792(6).
36. Ferrante R.J., et al. Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice. Society for Neuroscience Abstract Viewer and Itinerary Planner 2003, 2003. (abstract no. 130.7).
37. Fischer A., et al. Targeting the correct HDAC(s) to treat cognitive disorders. Trends in Pharmacological Sciences 2010, 31(12):605-617.
38. Flint J. Analysis of quantitative trait loci that influence animal behavior. Journal of Neurobiology 2003, 54(1):46-77.
39. Flint J. Mapping quantitative traits and strategies to find quantitative trait genes. Methods 2011, 53(2):163-174.
40. Fossale E., et al. Identification of a presymptomatic molecular phenotype in Hdh CAG knock-in mice. Human Molecular Genetics 2002, 11:2233-2241.
41. Fu X., et al. Estimating accuracy of RNA-Seq and microarrays with proteomics. BMC Genomics 2009, 10:9.
42. Gardian G., et al. Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease. Journal of Biological Chemistry 2005, 280(1):556-563.
43. Giles, P., et al., Gene expression changes over time and correlates with behaviour in the HdhQ150 mouse model of HD. Brain Research Bulletin, in this issue.
44. Glass M., et al. Delayed onset of Huntington's disease in mice in an enriched environment correlates with delayed loss of cannabinoid CB1 receptors. Neuroscience 2004, 123(1):207-212.
45. Gray M., et al. Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice. Journal of Neuroscience 2008, 28:6182-6195.
46. Guan Z.H., et al. Integration of long-term-memory-related synaptic plasticity involves bidirectional regulation of gene expression and chromatin structure. Cell 2002, 111(4):483-493.
47. Guan J.S., et al. HDAC2 negatively regulates memory formation and synaptic plasticity. Nature 2009, 459(7243):55-58.
48. Heng M.Y., et al. Longitudinal evaluation of the Hdh(CAG)150 knock-in murine model of Huntington's disease. The Journal of Neuroscience: The Official Journal of the Society for Neuroscience 2007, 27:8989-8998.
49. Heng M.Y., et al. Early autophagic response in a novel knock-in model of Huntington disease. Human Molecular Genetics 2010, 19:3702-3720.
50. Hockly E., et al. Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease. Proceedings of the National Academy of Sciences of the United States of America 2003, 100(4):2041-2046.
51. Hodges A., et al. Regional and cellular gene expression changes in human Huntington's disease brain. Human Molecular Genetics 2006, 15(6):965-977.
52. Hodges A., et al. Brain gene expression correlates with changes in behavior in the R6/1 mouse model of Huntington's disease. Genes Brain and Behavior 2008, 7(3):288-299.
53. Hodgson J.G., et al. A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron 1999, 23:181-192.
54. Hornshoj H., et al. Transcriptomic and proteomic profiling of two porcine tissues using high-throughput technologies. BMC Genomics 2009, 10:12.
55. Hovatta I., Barlow C. Molecular genetics of anxiety in mice and men. Annals of Medicine 2008, 40(2):92-109.
56. Huang C.C., et al. Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins. Somatic Cell and Molecular Genetics 1998, 24(4):217-233.
57. Ishiguro H., et al. Repeat instability occurs in mouse knock-in for a mutant Huntington's disease gene. Journal of Neuroscience Research 2001, 297:289-297.
58. Kilgore M., et al. Inhibitors of Class 1 histone deacetylases reverse contextual memory deficits in a mouse model of Alzheimer's disease. Neuropsychopharmacology 2010, 35(4):870-880.
59. Kita H., et al. Modulation of polyglutamine-induced cell death by genes identified by expression profiling. Human Molecular Genetics 2002, 11(19):2279-2287.
60. Kuhn A., et al. Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage. Human Molecular Genetics 2007, 16(15):1845-1861.
61. Lamprecht R., et al. Fear conditioning induces distinct patterns of gene expression in lateral amygdala. Genes, Brain and Behavior 2009, 8(8):735-743.
62. Levenson J.M., Sweatt J.D. Epigenetic mechanisms: a common theme in vertebrate and invertebrate memory formation. Cellular and Molecular Life Sciences 2006, 63(9):1009-1016.
63. Li H., et al. Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice. Human Molecular Genetics 1999, 8(7):1227-1236.
64. Licatalosi D.D., Darnell R.B. Applications of next-generation sequencing RNA processing and its regulation: global insights into biological networks. Nature Reviews Genetics 2010, 11(1):75-87.
65. Lin C.-H. Neurological abnormalities in a knock-in mouse model of Huntington's disease. Human Molecular Genetics 2001, 10:137-144.
66. Lu T., et al. Gene regulation and DNA damage in the ageing human brain. Nature 2004, 429(6994):883-891.
67. Luthi-Carter R., et al. Decreased expression of striatal signaling genes in a mouse model of Huntington's disease. Human Molecular Genetics 2000, 9(9):1259-1271.
68. Luthi-Carter R., et al. Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects. Human Molecular Genetics 2002, 11(17):1927-1937.
69. Luthi-Carter R., et al. Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain. Human Molecular Genetics 2002, 11(17):1911-1926.
70. Mangiarini L., et al. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 1996, 87:493-506.
71. Meade C.a., et al. Cellular localization and development of neuronal intranuclear inclusions in striatal and cortical neurons in R6/2 transgenic mice. The Journal of Comparative Neurology 2002, 449:241-269.
72. Menalled L.B., et al. Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice. Image (Rochester, N.Y.) 2002, 22:8266-8276.
73. Menalled L.B., et al. Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats. The Journal of Comparative Neurology 2003, 465:11-26.
74. Mihm M.J., et al. Cardiac dysfunction in the R6/2 mouse model of Huntington's disease. Neurobiology of disease 2007, 25:297-308.
75. Miller B.H., et al. Genetic regulation of behavioral and neuronal responses to fluoxetine. Neuropsychopharmacology 2007, 33(6):1312-1322.
76. Milnerwood A.J., et al. Early development of aberrant synaptic plasticity in a mouse model of Huntington's disease. Human Molecular Genetics 2006, 15(10):1690-1703.
77. Moffitt H., 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. PloS One 2009, 4:e8025.
78. Morris M.J., Karra A.S., Monteggia L.M. Histone deacetylases govern cellular mechanisms underlying behavioral and synaptic plasticity in the developing and adult brain. Behavioural Pharmacology 2010, 21(5-6):409-419.
79. Morton A.J., et al. Progressive formation of inclusions in the striatum and hippocampus of mice transgenic for the human Huntington's disease mutation. Journal of Neurocytology 2000, 29(9):679-702.
80. Morton A.J., et al. A combination drug therapy improves cognition and reverses gene expression changes in a mouse model of Huntington's disease. European Journal of Neuroscience 2005, 21(4):855-870.
81. Mulligan M.K., et al. Molecular profiles of drinking alcohol to intoxication in C57BL/6J mice. Alcoholism-Clinical and Experimental Research 2011, 35(4):659-670.
82. Nguyen H.P., et al. Age-dependent gene expression profile and protein expression in a transgenic rat model of Huntington's disease. Proteomics Clinical Applications 2008, 2(12):1638-1650.
83. Nie L., et al. Integrative analysis of transcriptomic and proteomic data: challenges, solutions and applications. Critical Reviews in Biotechnology 2007, 27(2):63-75.
84. Norris P.J., et al. Decreased neuronal nitric oxide synthase messenger RNA and somatostatin messenger RNA in the striatum of Huntington's disease. Neuroscience 1996, 72(4):1037-1047.
85. Nucifora F.C., et al. Interference by Huntingtin and atrophin-1 with CBP-mediated transcription leading to cellular toxicity. Science 2001, 291(5512):2423-2428.
86. Pallos J., et al. Inhibition of specific HDACs and sirtuins suppresses pathogenesis in a Drosophila model of Huntington's disease. Human Molecular Genetics 2008, 17(23):3767-3775.
87. Pan Q., et al. Deep surveying of alternative splicing complexity in the human transcriptome by high-throughput sequencing. Nature Genetics 2008, 40(12):1413-1415.
88. Park C.C., et al. Dual spatial maps of transcript and protein abundance in the mouse brain. Expert Review of Proteomics 2009, 6(3):243-249.
89. Ponder C.A., et al. Rapid selection response for contextual fear conditioning in a cross between C57BL/6J and A/J: Behavioral, QTL and gene expression analysis. Behavior Genetics 2008, 38(3):277-291.
90. Ravache M., et al. Transcriptional Activation of REST by Sp1 in Huntington's disease models. PLoS One 2010, 5(12). (article no.: e14311).
91. Reddy P.H., et al. Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA. Nature genetics 1998, 20:198-202.
92. Richfield E.K., et al. Reduced expression of preproenkephalin in striatal neurons from huntingtons-disease patients. Annals of Neurology 1995, 37(3):335-343.
93. Runne H., et al. Analysis of potential transcriptomic biomarkers for Huntington's disease in peripheral blood. Proceedings of the National Academy of Sciences of the United States of America 2007, 104(36):14424-14429.
94. Runne H., et al. Dysregulation of gene expression in primary neuron models of Huntington's disease shows that polyglutamine-related effects on the striatal transcriptome may not be dependent on brain circuitry. Journal of Neuroscience 2008, 28(39):9723-9731.
95. Sadri-Vakili G., et al. Huntingtin inclusions do not down-regulate specific genes in the R6/2 Huntington's disease mouse. European Journal of Neuroscience 2006, 23(12):3171-3175.
96. Sadri-Vakili G., et al. Histones associated with downregulated genes are hypo-acetylated in Huntington's disease models. Human Molecular Genetics 2007, 16(11):1293-1306.
97. Sathasivam K., et al. Formation of polyglutamine inclusions in non-CNS tissue. Human Molecular Genetics 1999, 8:813-822.
98. Savas J.N., et al. A role for Huntington disease protein in dendritic RNA granules. Journal of Biological Chemistry 2010, 285(17):13142-13153.
99. Schilling G., et al. Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin. Human Molecular Genetics 1999, 8:397-407.
100. Shelbourne P. A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice. Human Molecular Genetics 1999, 8:763-774.
101. Sherrin T., et al. Region specific gene expression profile in mouse brain after chronic corticotropin releasing factor receptor 1 activation: the novel role for diazepam binding inhibitor in contextual fear conditioning. Neuroscience 2009, 162(1):14-22.
102. Shimohata T., et al. Expanded polyglutamine stretches interact with TAF(II)130, interfering with CREB-dependent transcription. Nature Genetics 2000, 26(1):29-36.
103. Shyu A.B., Wilkinson M.F., van Hoof A. Messenger RNA regulation: to translate or to degrade. Embo Journal 2008, 27(3):471-481.
104. Sipione S., et al. Early transcriptional profiles in huntingtin-inducible striatal cells by microarray analyses. Human Molecular Genetics 2002, 11(17):1953-1965.
105. Slow E.J. Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Human Molecular Genetics 2003, 12:1555-1567.
106. Slow E.J., et al. Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions. Proceedings of the National Academy of Sciences of the United States of America 2005, 102:11402-11407.
107. Spires T.L., et al. Abnormal dendritic morphology in R6/1 Huntington's disease mice: effects of environmental enrichment. Society for Neuroscience Abstract Viewer and Itinerary Planner 2003, (abstract no. 130.6).
108. Spires T.L., et al. Environmental enrichment rescues protein deficits in a mouse model of Huntington's disease, indicating a possible disease mechanism. Journal of Neuroscience 2004, 24(9):2270-2276.
109. Steffan J.S., et al. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proceedings of the National Academy of Sciences of the United States of America 2000, 97(12):6763-6768.
110. Steffan J.S., et al. Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila. Nature 2001, 413(6857):739-743.
111. Strand A.D., et al. Expression profiling of Huntington's disease models suggests that brain-derived neurotrophic factor depletion plays a major role in striatal degeneration. Journal of Neuroscience 2007, 27(43):11758-11768.
112. Surget A., et al. Corticolimbic transcriptome changes are state-dependent and region-specific in a rodent model of depression and of antidepressant reversal. Neuropsychopharmacology 2008, 34(6):1363-1380.
113. Tabrizi S.J., et al. Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis. Lancet Neurology 2011, 10(1):31-42.
114. Tanaka Y., et al. Progressive phenotype and nuclear accumulation of an amino-terminal cleavage fragment in a transgenic mouse model with inducible expression of full-length mutant huntingtin. Neurobiology of Disease 2006, 21:381-391.
115. Thiriet N., et al. Environmental enrichment during adolescence regulates gene expression in the striatum of mice. Brain Research 2008, 1222:31-41.
116. Thomas E.A., et al. The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice. Proceedings of the National Academy of Sciences of the United States of America 2008, 105(40):15564-15569.
117. Thomas E.A., et al. In vivo cell-autonomous transcriptional abnormalities revealed in mice expressing mutant huntingtin in striatal but not cortical neurons. Human Molecular Genetics 2011, 20(6):1049-1060.
118. Usdin M.T., et al. Impaired synaptic plasticity in mice carrying the Huntington's disease mutation. Neurobiology 1999, 8:839-846.
119. Van Raamsdonk J.M., et al. Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease. The Journal of neuroscience: the official journal of the Society for Neuroscience 2005, 25:4169-4180.
120. Van Raamsdonk J.M., et al. Body weight is modulated by levels of full-length huntingtin. Human molecular genetics 2006, 15:1513-1523.
121. Vecsey C.G., et al. Histone deacetylase inhibitors enhance memory and synaptic plasticity via CREB: CBP-dependent transcriptional activation. Journal of Neuroscience 2007, 27(23):6128-6140.
122. Vonsattel J.P., et al. Neuropathological classification of Huntingtons-disease. Journal of Neuropathology and Experimental Neurology 1985, 44(6):559-577.
123. Wang E.T., et al. Alternative isoform regulation in human tissue transcriptomes. Nature (London) 2008, 456(7221):470-476.
124. Waters K.M., Pounds J.G., Thrall B.D. Data merging for integrated microarray and proteomic analysis. Briefings in Functional Genomics & Proteomics 2006, 5(4):261-272.
125. Weake V.M., Workman J.L. Inducible gene expression: diverse regulatory mechanisms. Nature Reviews Genetics 2010, 11(6):426-437.
126. Wheeler V. Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse. Human Molecular Genetics 1999, 8:115-122.
127. Wheeler V.C. Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice. Human Molecular Genetics 2000, 9:503-513.
128. Wheeler V.C., et al. Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice. Human Molecular Genetics 2002, 11:633-640.
129. White J.K., Auerbach W., Duyao M.P., Vonsatter J.-P., Gusella J.F., joyner A.L., MacDonald M.E. Huntingtin is required for neurogenesis and is not impaired by the Huntingtin's disease CAG expansion. Nature Genetics 1997, 17:404-410.
130. Yamamoto A., Lucas J.J., Hen R. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell 2000, 101:57-66.
131. Yu Z.-x., et al. Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease. Control 2003, 23:2193-2202.
132. Zheng Z.M. Regulation of alternative RNA splicing by exon definition and exon sequences in viral and mammalian gene expression. Journal of Biomedical Science 2004, 11(3):278-294.
133. Zuccato C., et al. Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes. Nature Genetics 2003, 35(1):76-83.
134. Zuccato C., Valenza M., Cattaneo E. Molecular mechanisms and potential therapeutical targets in Huntington's disease. Physiological Reviews 2010, 90:905-981.
135. Zucker B., et al. Transcriptional dysregulation in striatal projection- and interneurons in a mouse model of Huntington's disease: neuronal selectivity and potential neuroprotective role of HAP1. Human Molecular Genetics 2005, 14(2):179-189.