Early deficits in motor coordination and cognitive dysfunction in a mouse model of the neurodegenerative lysosomal storage disorder, Sandhoff disease

Behavioural Brain Research

Volumn 193, Issue 2, 2008, Pages 315-319

  Gulinello, Maria ^a   Chen, Fengying ^a   Dobrenis, Kostantin ^a  

^a ALBERT EINSTEIN COLLEGE OF MEDICINE   (United States)

Author keywords

Balance beam; Memory; Motor coordination; Mouse model; Novel object recognition test; Open field; Rotarod; Sandhoff disease

Indexed keywords

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; COGNITION; COGNITIVE DEFECT; CONTROLLED STUDY; DEGENERATIVE DISEASE; DISEASE MODEL; MEMORY DISORDER; MOTOR COORDINATION; MOTOR DYSFUNCTION; MOUSE; NONHUMAN; PRIORITY JOURNAL; PSYCHOLOGIC TEST; SANDHOFF DISEASE; SENSORIMOTOR FUNCTION; SHORT TERM MEMORY; WORKING MEMORY;

ANIMALS; BETA-HEXOSAMINIDASE BETA CHAIN; COGNITION; COGNITION DISORDERS; LYSOSOMAL STORAGE DISEASES; MEMORY DISORDERS; MEMORY, SHORT-TERM; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT; MOTOR ACTIVITY; ROTAROD PERFORMANCE TEST; SANDHOFF DISEASE;

EID: 48749101427     PISSN: 01664328     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.bbr.2008.06.016     Document Type: Article

References (28)

1. Andersson U., Smith D., Jeyakumar M., Butters T.D., Cortina Borja M., Dwek R.A., et al. Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease. Neurobiol Dis 16 (2004) 506-515
2. Bourgoin C., Emiliani C., Kremer E.J., Gelot A., Tancini B., Gravel R.A., et al. Widespread distribution of beta-hexosaminidase activity in the brain of a Sandhoff mouse model after coinjection of adenoviral vector and mannitol. Gene Ther 10 (2003) 1841-1849
3. Buitrago M.M., Schulz J.B., Dichgans J., and Luft A.R. Short and long-term motor skill learning in an accelerated rotarod training paradigm. Neurobiol Learn Mem 81 (2004) 211-216
4. Cachon-Gonzalez M.B., Wang S.Z., Lynch A., Ziegler R., Cheng S.H., Cox T.M., et al. Effective gene therapy in an authentic model of Tay-Sachs-related diseases. Proc Natl Acad Sci USA 103 (2006) 10373-10378
5. Choleris E., Thomas A.W., Kavaliers M., and Prato F.S. A detailed ethological analysis of the mouse open field test: effects of diazepam, chlordiazepoxide and an extremely low frequency pulsed magnetic field. Neurosci Biobehav Rev 25 (2001) 235-260
6. Clark R.E., Zola S.M., and Squire L.R. Impaired recognition memory in rats after damage to the hippocampus. J Neurosci 20 (2000) 8853-8860
7. Denny C.A., Alroy J., Pawlyk B.S., Sandberg M.A., d'Azzo A., and Seyfried T.N. Neurochemical, morphological, and neurophysiological abnormalities in retina of Sandhoff and GM1 gangliosidosis mice. J Neurochem 101 (2007) 1294-1302
8. Denny C.A., Kasperzyk J.L., Gorham K.N., Bronson R.T., and Seyfried T.N. Influence of caloric restriction on motor behavior, longevity, and brain lipid composition in Sandhoff disease mice. J Neurosci Res 83 (2006) 1028-1038
9. Ennaceur A., and Meliani K. A new one-trial test for neurobiological studies of memory in rats. III. Spatial vs. non-spatial working memory. Behav Brain Res 51 (1992) 83-92
10. Frey L.C., Ringel S.P., and Filley C.M. The natural history of cognitive dysfunction in late-onset GM2 gangliosidosis. Arch Neurol 62 (2005) 989-994
11. Gravel R.A., Clarke J.T.R., Kaback M.M., Mahuran D., Sandhoff K., and Suzuki K. The GM2 gangliosidoses. In: Scriver C.R., Beaudet A.L., Sly W.S., and Valle D. (Eds). The metabolic and molecular bases of inherited disease. VII edition (2001), McGraw-Hill, New York 3827-3876
12. Irwin S. Comprehensive observational assessment. Ia. A systematic, quantitative procedure for assessing the behavioral and physiologic state of the mouse. Psychopharmacologia 13 (1968) 222-257
13. Jeyakumar M., Butters T.D., Cortina Borja M., Hunnam V., Proia R.L., Perry V.H., et al. Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin. Proc Natl Acad Sci USA 96 (1999) 6388-6393
14. Jeyakumar M., Norflus F., Tifft C.J., Corina-Borja M., Butters T.D., Proia R.L., et al. Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation. Blood 97 (2001) 327-329
15. Jeyakumar M., Smith D.A., Williams I.M., Cortina Borja M., Neville D.C.A., Butters T.D., et al. NSAIDS increase survival in the Sandhoff disease mouse: synergy with N-butyldeoxynojirimycin. Ann Neurol 56 (2004) 642-649
16. Jeyakumar M., Thomas R., Elliot-Smith E., Smith D.A., van der Spoel A.C., d'Azzo A., et al. Central nervous system inflammation is a hallmark of pathogenesis in mouse models of GM1 and GM2 gangliosidosis. Brain 126 (2003) 974-987
17. Kurylo D.D., Corkin S., Rizzo III J.F., and Growden J.H. Greater relative impairment of object recognition than of visuospatial abilities in Alzheimer's disease. Neuropsychology 10 (1996) 74-81
18. Kyrkanides S., Miller J.H., Brouxhon S.M., Olschowka J.A., and Federoff H.J. Beta-hexosaminidase lentiviral vectors: transfer into the CNS via systemic administration. Brain Res Mol Brain Res 133 (2005) 286-298
19. Lalonde R., and Strazielle C. Motor coordination, exploration, and spatial learning in a natural mouse mutation (nervous) with Purkinje cell degeneration. Behav Genet 33 (2003) 59-66
20. Lee J.P., Jeyakumar M., Gonzalez R., Takahashi H., Lee P.J., Baek R.C., et al. Stem cells act through multiple mechanisms to benefit mice with neurodegenerative metabolic disease. Nat Med 13 (2007) 439-447
21. MacQueen G.M., Rosebush P.I., and Mazurek M.F. Neuropsychiatric aspects of the adult variant of Tay-Sachs disease. J Neuropsychiatry Clin Neurosci 10 (1998) 10-19
22. Norflus F., Tifft C.J., McDonald M.P., Goldstein G., Crawley J.N., Hoffmann A., et al. Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice. J Clin Invest 101 (1998) 1881-1888
23. Phaneuf D., Wakamatsu N., Huang J.Q., Borowski A., Peterson A.C., Fortunato S.R., et al. Dramatically different phenotypes in mouse models of human Tay-Sachs and Sandhoff diseases. Hum Mol Genet 5 (1996) 1-14
24. Rogers D.C., Peters J., Martin J.E., Ball S., Nicholson S.J., Witherden A.S., et al. SHIRPA, a protocol for behavioral assessment: validation for longitudinal study of neurological dysfunction in mice. Neurosci Lett 306 (2001) 89-92
25. Sango K., Yamanaka S., Hoffmann A., Okuda Y., Grinberg A., Westphal H., et al. Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism. Nat Genet 11 (1995) 170-176
26. Stanley J.L., Lincoln R.J., Brown T.A., McDonald L.M., Dawson G.R., and Reynolds D.S. The mouse beam walking assay offers improved sensitivity over the mouse rotarod in determining motor coordination deficits induced by benzodiazepines. J Psychopharmacol 19 (2005) 221-227
27. Wu Y.P., and Proia R.L. Deletion of macrophage-inflammatory protein 1alpha retards neurodegeneration in Sandhoff disease mice. Proc Natl Acad Sci USA 101 (2004) 8425-8430
28. Yamaguchi A., Katsuyama K., Nagahama K., Takai T., Aoki I., and Yamanaka S. Possible role of autoantibodies in the pathophysiology of GM2 gangliosidoses. J Clin Invest 113 (2004) 200-208