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- Title
Deletion in Catna2, encoding αN-catenin, causes cerebellar and hippocampal lamination defects and impaired startle modulation.
- Authors
Park, Chankyu; Falls, William; Finger, Jacqueline H.; Longo-Guess, Chantal M.; Ackerman, Susan L.
- Abstract
Mice homozygous for the cerebellar deficient folia (cdf) mutation are ataxic and have cerebellar hypoplasia and abnormal lobulation of the cerebellum. In the cerebella of cdf/cdf homozygous mice, approximately 40% of Purkinje cells are located ectopically in the white matter and inner granule-cell layer. Many hippocampal pyramidal cells are scattered in the plexiform layers, and those that are correctly positioned are less densely packed than are cells in wild-type mice. We show that fear conditioning and prepulse inhibition of the startle response are also disrupted in cdf/cdf mice. We identify a deletion on chromosome 6 that removes approximately 150 kb in the cdf critical region. The deletion includes part of Catna2, encoding αN-catenin, a protein that links the classical cadherins to the neuronal cytoskeleton. Expression of a Catna2 transgene in cdf/cdf mice restored normal cerebellar and hippocampal morphology, prepulse inhibition and fear conditioning. The findings suggest that catenin-cadherin celladhesion complexes are important in cerebellar and hippocampal lamination and in the control of startle modulation.
- Subjects
CEREBELLUM degeneration; MICE; PURKINJE cells
- Publication
Nature Genetics, 2002, Vol 31, Issue 3, p279
- ISSN
1061-4036
- Publication type
Article
- DOI
10.1038/ng908