αT-catenin in restricted brain cell types and its potential connection to autism

Journal of Molecular Psychiatry - Tập 4 - Trang 1-13 - 2016
Stephen Sai Folmsbee1,2, Douglas R. Wilcox3,4,2, Koen Tyberghein5,6, Pieter De Bleser5,6, Warren G. Tourtellotte7,8,2, Jolanda van Hengel5,6,9, Frans van Roy5,6, Cara J. Gottardi1,10,2
1Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, USA
2The Driskill Graduate Training Program in Life Sciences, Northwestern University Feinberg School of Medicine, Chicago, USA
3Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, USA
4Department of Microbiology-Immunology, Northwestern University, Feinberg School of Medicine, Chicago, USA
5Department of Biomedical Molecular Biology, Molecular Cell Biology Unit, Ghent University, Ghent, Belgium
6Inflammation Research Center, Flanders Institute for Biotechnology (VIB), Ghent, Belgium
7Department of Pathology, Northwestern University Feinberg School of Medicine, Chicago, USA
8Department of Neurology, Northwestern University Feinberg School of Medicine, Chicago, USA
9Department of Basic Medical Sciences, Faculty of Medicine and Health Sciences, Ghent University, Ghent, Belgium
10Department of Cellular and Molecular Biology, Northwestern University Feinberg School of Medicine, Chicago, USA

Tóm tắt

Recent genetic association studies have linked the cadherin-based adherens junction protein alpha-T-catenin (αT-cat, CTNNA3) with the development of autism. Where αT-cat is expressed in the brain, and how its loss could contribute to this disorder, are entirely unknown. We used the αT-cat knockout mouse to examine the localization of αT-cat in the brain, and we used histology and immunofluorescence analysis to examine the neurobiological consequences of its loss. We found that αT-cat comprises the ependymal cell junctions of the ventricles of the brain, and its loss led to compensatory upregulation of αE-cat expression. Notably, αT-cat was not detected within the choroid plexus, which relies on cell junction components common to typical epithelial cells. While αT-cat was not detected in neurons of the cerebral cortex, it was abundantly detected within neuronal structures of the molecular layer of the cerebellum. Although αT-cat loss led to no overt differences in cerebral or cerebellar structure, RNA-sequencing analysis from wild type versus knockout cerebella identified a number of disease-relevant signaling pathways associated with αT-cat loss, such as GABA-A receptor activation. These findings raise the possibility that the genetic associations between αT-cat and autism may be due to ependymal and cerebellar defects, and highlight the potential importance of a seemingly redundant adherens junction component to a neurological disorder.

Tài liệu tham khảo

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