title: Early deficits in motor coordination and cognitive dysfunction in a mouse model of the neurodegenerative lysosomal storage disorder, Sandhoff disease.
abstract: Mouse models of lysosomal storage diseases, including Sandhoff disease, are frequently employed to test therapies directed at the central nervous system. We backbred such mice and conducted a behavioral test battery which included sensorimotor and cognitive assessments. This is the first report of short-term memory deficits in a murine model of Sandhoff disease. We also document early onset of motor deficits using the balance beam test.
authors.affiliation: Behavioral Core Facility, Dominick S. Purpura Department of Neuroscience, Albert Einstein College of Medicine of Yeshiva University, Bronx, NY, USA.
authors.forename: Maria
authors.initials: M
authors.lastname: Gulinello
authors.forename: Fengying
authors.initials: F
authors.lastname: Chen
authors.forename: Kostantin
authors.initials: K
authors.lastname: Dobrenis
mesh_terms: D000818:Animals; D003071:Cognition / Q000502:physiology; D003072:Cognition Disorders / Q000235:genetics / Q000503:physiopathology*; D016464:Lysosomal Storage Diseases / Q000235:genetics / Q000503:physiopathology; D008569:Memory Disorders / Q000235:genetics / Q000503:physiopathology*; D008570:Memory, Short-Term / Q000502:physiology; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D009043:Motor Activity / Q000502:physiology*; D045442:Rotarod Performance Test / Q000379:methods; D012497:Sandhoff Disease / Q000235:genetics / Q000503:physiopathology*; D054821:beta-Hexosaminidase beta Chain / Q000235:genetics
chemical_list: D054821:beta-Hexosaminidase beta Chain