Standardization and statistical approaches to therapeutic trials in the R6/2 mouse

Brain Research Bulletin

Volumn 61, Issue 5, 2003, Pages 469-479

  Hockly, Emma ^a   Woodman, Benjamin ^a   Mahal, Amarbirpal ^a   Lewis, Cathryn M ^a   Bates, Gillian ^a  

^a KING'S COLLEGE LONDON   (United Kingdom)

Author keywords

Behavior; Huntington's disease; Mouse models; Preclinical trials; Statistical analysis

Indexed keywords

ANALYTIC METHOD; ANIMAL EXPERIMENT; ARTICLE; BIOLOGICAL MODEL; CONTROLLED STUDY; DATA ANALYSIS; ENVIRONMENTAL FACTOR; FEMALE; GENETIC VARIABILITY; GRIP STRENGTH; HUNTINGTON CHOREA; MALE; MOUSE; MOUSE STRAIN; NONHUMAN; PHENOTYPIC VARIATION; PRIORITY JOURNAL; STANDARDIZATION; STATISTICAL ANALYSIS; TRANSGENIC MOUSE; WEIGHT REDUCTION;

EID: 0141678246     PISSN: 03619230     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0361-9230(03)00185-0     Document Type: Article

References (29)

1. G.P. Bates, L. Jones. Huntington's disease. Oxford: Oxford University Press; 2002.
2. Becher M.W., Kotzuk J.A., Sharp A.H., Davies S.W., Bates G.P., Price D.L.et al. Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length. Neurobiol. Dis. 4:1998;387-397.
3. Benjamini Y., Drai D., Elmer G., Kafkafi N., Golani I. Controlling the false discovery rate in behavior genetics research. Behav. Brain Res. 125:2001;279-284.
4. Bonferroni C.E. Teoria statistica delle classi e calcolo delle probabilita. Pubblicazioni del R Istituto Superiore di Scienze Economiche e Commerciali di Firenze. 8:1936;3-62.
5. Carter R.J., Hunt M.J., Morton A.J. Environmental stimulation increases survival in mice transgenic for exon 1 of the Huntington's disease gene. Mov. Disord. 15:2000;925-937.
6. Carter R.J., Lione L.A., Humby T., Mangiarini L., Mahal A., Bates G.P.et al. Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation. J. Neurosci. 19:1999;3248-3257.
7. Clark H.B., Burright E.N., Yunis W.S., Larson S., Wilcox C., Hartman B. et al. Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations. J. Neurosci. 17:1997;7385-7395.
8. Davies S.W., Turmaine M., Cozens B.A., DiFiglia M., Sharp A.H., Ross C.A.et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell. 90:1997;537-548.
9. DiFiglia M., Sapp E., Chase K.O., Davies S.W., Bates G.P., Vonsattel J.P.et al. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science. 277:1997;1990-1993.
10. Fisher RA. Statistical methods for research workers. 4th ed. London: Oliver and Boyd; 1932.
11. Gutekunst C.A., Li S.H., Yi H., Mulroy J.S., Kuemmerle S., Jones R.et al. Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. J. Neurosci. 19:1999;2522-2534.
12. Hochberg Y., Benjamini Y. More powerful procedures for multiple significance testing. Stat. Med. 9:1990;811-818.
13. Hockly E., Cordery P.M., Woodman B., Mahal A., van Dellen A., Blakemore C.et al. Environmental enrichment slows disease progression in R6/2 Huntington's disease mice. Ann. Neurol. 51:2002;235-242.
14. Hockly E., Richon V.M., Woodman B., Smith D.L., Zhou X., Rosa E.et al. Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease. Proc. Natl. Acad. Sci. USA. 100:2003;2041-2046.
15. Hodgson J.G., Agopyan N., Gutekunst C.A., Leavitt B.R., LePiane F., Singaraja R.et al. A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron. 23:1999;181-192.
16. Kempermann G., Brandon E.P., Gage F.H. Environmental stimulation of 129/SvJ mice causes increased cell proliferation and neurogenesis in the adult dentate gyrus. Curr. Biol. 8:1998;939-942.
17. Levine M.S., Klapstein G.J., Koppel A., Gruen E., Cepeda C., Vargas M.E.et al. Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease. J. Neurosci. Res. 58:1999;515-532.
18. Li H., Li S.H., Cheng A.L., Mangiarini L., Bates G.P., Li X.J. Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice. Hum. Mol. Genet. 8:1999;1227-1236.
19. Lin C.H., Tallaksen-Greene S., Chien W.M., Cearley J.A., Jackson W.S., Crouse A.B.et al. Neurological abnormalities in a knock-in mouse model of Huntington's disease. Hum. Mol. Genet. 10:2001;137-144.
20. Lione L.A., Carter R.J., Hunt M.J., Bates G.P., Morton A.J., Dunnett S.B. Selective discrimination learning impairments in mice expressing the human Huntington's disease mutation. J. Neurosci. 19:1999;10428-10437.
21. Mangiarini L., Sathasivam K., Mahal A., Mott R., Seller M., Bates G.P. Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation. Nat. Genet. 15:1997;197-200.
22. Mangiarini L., Sathasivam K., Seller M., Cozens B., Harper A., Hetherington C.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. 87:1996;493-506.
23. Myers RH, Marans KS, MacDonald ME. Huntington's disease. In: Wells RD, Warren ST, editors. Genetic instabilities and hereditary neurological diseases. San Diego, Academic Press; 1998. p. 301-23.
24. Reddy P.H., Charles V., Williams M., Miller G., Whetsell W.O. Jr., Tagle D.A. Transgenic mice expressing mutated full-length HD cDNA: a paradigm for locomotor changes and selective neuronal loss in Huntington's disease. Philos. Trans. R. Soc. Lond. B. Biol. Sci. 354:1999;1035-1045.
25. Shelbourne P.F., Killeen N., Hevner R.F., Johnston H.M., Tecott L., Lewandoski M.et al. A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice. Hum. Mol. Genet. 8:1999;763-774.
26. Stephens M.A. EDF statistics for goodness of fit and some comparisons. J. Am. Stat. Assoc. 69:1974;730-737.
27. van Praag H., Kempermann G., Gage F.H. Neural consequences of environmental enrichment. Nat. Rev. Neurosci. 1:2000;191-198.
28. Wheeler V.C., Auerbach W., White J.K., Srinidhi J., Auerbach A., Ryan A.et al. Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse. Hum. Mol. Genet. 8:1999;115-122.
29. Yamamoto A., Lucas J.J., Hen R. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell. 101:2000;57-66.