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The Journal of Neuroscience, July 15, 1998, 18(14):5508-5516

Mice Lacking Ataxin-1 Display Learning Deficits and Decreased Hippocampal Paired-Pulse Facilitation

Antoni Matilla1, Erik D. Roberson2, Sandro Banfi1, Joanella Morales3, Dawna L. Armstrong4, Eric N. Burright7, Harry T. Orr7, John D. Sweatt2, Huda Y. Zoghbi1, 2, 3, 6, and Martin M. Matzuk3, 4, 5

Departments of 1 Pediatrics, 2 Neuroscience, 3 Molecular and Human Genetics, 4 Pathology, and 5 Cell Biology, and 6 Howard Hughes Medical Institute, Baylor College of Medicine, Houston, Texas 77030, and 7 University of Minnesota, Minneapolis, Minnesota 55455

Spinocerebellar ataxia type 1 (SCA1) is a neurodegenerative disorder characterized by ataxia, progressive motor deterioration, and loss of cerebellar Purkinje cells. To investigate SCA1 pathogenesis and to gain insight into the function of the SCA1 gene product ataxin-1, a novel protein without homology to previously described proteins, we generated mice with a targeted deletion in the murine Sca1 gene. Mice lacking ataxin-1 are viable, fertile, and do not show any evidence of ataxia or neurodegeneration. However, Sca1 null mice demonstrate decreased exploratory behavior, pronounced deficits in the spatial version of the Morris water maze test, and impaired performance on the rotating rod apparatus. Furthermore, neurophysiological studies performed in area CA1 of the hippocampus reveal decreased paired-pulse facilitation in Sca1 null mice, whereas long-term and post-tetanic potentiations are normal. These findings demonstrate that SCA1 is not caused by loss of function of ataxin-1 and point to the possible role of ataxin-1 in learning and memory.

Key words: spinocerebellar ataxia type 1; ataxin-1; neurobehavior; hippocampus; cerebellum; paired-pulse facilitation

Copyright © 1998 Society for Neuroscience  0270-6474/98/18145508-09$05.00/0


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