Jagged1 is a ligand for members of the Notch family of receptors. Mutations in the human JAG1 gene are the major cause of Alagille syndrome, an autosomal dominant disorder affecting the liver, heart, eye, skeleton, kidneys, and craniofacial structures. Although expressed throughout mammalian embryonic development and in the adult, the function of Jagged1 in the central nervous system is not clear. Jagged1 is broadly expressed in the cerebellum suggesting an important role in Notch signaling. In order to address the function of Jagged1 in the mouse central nervous system, we have inactivated the Jag1 gene in the cerebellar primordium at mid-embryogenesis. Loss of Jagged1 results in aberrant granule cell migration and ectopic differentiation in the external germinal layer and molecular layer of the early postnatal cerebellum. We show that Bergmann glia in the cerebellum lose contact to the pial surface and have stunted processes. In vitro analysis revealed a depletion of Bergmann glia in the Jagged1 mutant mice. Our findings suggest that Jagged1 plays a role in cell fate specification and survival in the cerebellum.

Page 1. Fax +41 61 306 12 34 E-Mail karger@karger.ch www.karger.com 169 Author Index Vol. 28, No. 1–2, 2006 Alexson, TO 34 Arboleda-Velasquez, JF 5 Artavanis-Tsakonas, S. 5 Beglopoulos, V. 102 Bernstein, A. 34 Bongarzone, ER 81 Chapman, G. 118 Coles, BL 34 Dang, L. 58 de Planell, M. 81 de Vellis, J. 81 Dohda, T. 118 Fishell, G. 49 Gaiano, N. 58 Galbiati, F. 81 Gennarini, G. 25 Givogri, MI 81 Gridley, T. 49 Gritti, A. 81 Gumuscu, B. 128 Gustafsson, MV 118 Hansson, EM 118 Hartman, BH 128 Hatakeyama, J. 92 Hitoshi, S. 34 Hu, Q.-D. 25 Kageyama, R. 92 Kopan, R. 102 Krautler, N. 70 Lendahl, U. 118 Louvi, A. 5 Ma, Q.-H. 25 Mantei, N. 70 Mason, HA 49 © 2006 S. Karger AG, Basel Accessible online at: www.karger.com/dne Meletis, K. 118 Muhr, J. 118 Nakao, K. 142 Nelson, BR 128 Okano, H. 142 Petersen, PH 156 Rakowiecki, SM 49 Reh, TA 128 …