Sweet’s syndrome in a neonate with non-B54 types of human leukocyte antigen.pdfVIP

Mol Neurobiol DOI 10.1007/s12035-015-9450-5 Early Disruption of Extracellular Pleiotrophin Distribution Alters Cerebellar Neuronal Circuit Development and Function M. M. Hamza 1,2 S. A. Rey3 P. Hilber4 A. Arabo 5 T. Collin3 D. Vaudry 1,2 D. Burel 1,2 Received: 20 July 2015 /Accepted: 16 September 2015 # The Author(s) 2015. This article is published with open access at S Abstract The cerebellum is a structure of the central nervous increase in the frequency of spontaneous excitatory postsyn- system involved in balance, motor coordination, and volun- aptic currents in PTN-treated mice, associated with a decrease tary movements. The elementary circuit implicated in the con- of climbing fiber innervations and an abnormal perisomatic trol of locomotion involves Purkinje cells, which receive ex- localization of the parallel fiber contacts. At adulthood, PTN- citatory inputs from parallel and climbing fibers, and are reg- treated mice exhibit coordination impairment on the rotarod ulated by cerebellar interneurons. In mice as in human, the test associated with an alteration of the synchronization gait. cerebellar cortex completes its development mainly after birth Altogether these histological, electrophysiological, and be- with the migration, differentiation, and synaptogenesis of havior data reveal that an early ECM disruption of PTN com- granule cells. These cellular events are under the control of position induces short- and long-term defaults in the establish- numerous extracellular matrix molecules including ment of proper functional cerebellar circuit. pleiotrophin (PTN). This cytokine has been shown to regulate the morphogenesis of Purkinje cells ex vivo and in vivo via its Keywords Pleiotrophin . Cerebellum . Neurodevelopment . receptor PTPζ. Since Purkinje ce