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

• Published in: Behavioural brain research Vol. 193; no. 2; pp. 315 - 319
• Main Authors: Gulinello, Maria, Chen, Fengying, Dobrenis, Kostantin
• Format: Journal Article
• Language: English
• Published: Shannon Elsevier B.V 21.11.2008; Elsevier Science
• Subjects: Animals; Balance beam; Behavioral psychophysiology; beta-Hexosaminidase beta Chain - genetics; Biological and medical sciences; Cognition - physiology; Cognition Disorders - genetics; Cognition Disorders - physiopathology; Errors of metabolism; Fundamental and applied biological sciences. Psychology; Lipids (lysosomal enzyme disorders, storage diseases); Lysosomal Storage Diseases - genetics; Lysosomal Storage Diseases - physiopathology; Medical sciences; Memory; Memory Disorders - genetics; Memory Disorders - physiopathology; Memory, Short-Term - physiology; Metabolic diseases; Mice; Mice, Inbred C57BL; Mice, Knockout; Motor Activity - physiology; Motor coordination; Mouse model; Novel object recognition test; Open field; Psychology. Psychoanalysis. Psychiatry; Psychology. Psychophysiology; Rotarod; Rotarod Performance Test - methods; Sandhoff disease; Sandhoff Disease - genetics; Sandhoff Disease - physiopathology; Balance beam; Novel object recognition test; Rotarod; Memory; Mouse model; Motor coordination; Open field; Sandhoff disease; Animal model; Gangliosidosis; Cognitive disorder; Lipids; Enzymopathy; Sandhoff disease;Mouse model;Novel object recognition test;Rotarod;Motor coordination;Balance beam;Open field;Memory; Lipoidosis; Motricity; Nervous system diseases; Rodentia; Metabolic diseases; Lysosomal storage disease; Motor control; Muscular coordination; Genetic disease; Cerebral disorder; Vertebrata; Mammalia; Mouse; Animal; Central nervous system disease; Early; Recognition
• ISSN: 0166-4328; 1872-7549
• DOI: 10.1016/j.bbr.2008.06.016

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.