Matilla, A;
Roberson, ED;
Banfi, S;
Morales, J;
Armstrong, DL;
Burright, ENS;
J D Orr, HT;
... Matzuk, M; + view all
(1998)
Mice lacking ataxin-1 display learning deficits and decreased hippocampal paired-pulse facilitation.
Journal of Neuroscience
, 18
(14)
5508 - 5516.
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Abstract
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.
| Type: | Article |
|---|---|
| Title: | Mice lacking ataxin-1 display learning deficits and decreased hippocampal paired-pulse facilitation. |
| Open access status: | An open access version is available from UCL Discovery |
| Language: | English |
| Additional information: | This work is licensed under the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 Unported License. The license allows you to copy, distribute, and transmit the work, as well as adapting it. However, you must attribute the work to the author (but not in any way that suggests that they endorse you or your use of the work), and cannot use the work for commercial purposes without prior permission of the author. If you alter or build upon this work, you can distribute the resulting work only under the same or similar license to this one. To view a copy of this license, visit http://creativecommons.org/licenses/by-nc-sa/3.0/ or send a letter to Creative Commons, 444 Castro Street, Suite 900, Mountain View, California, 94041, USA. |
| Keywords: | SCA1, ataxin-1, hippocampus, cerebellum, knockout |
| UCL classification: | UCL |
| URI: | https://discovery.ucl.ac.uk/id/eprint/182105 |
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