PT - JOURNAL ARTICLE AU - Corradi, Anna AU - Croci, Laura AU - Broccoli, Vania AU - Zecchini, Silvia AU - Previtali, Stefano AU - Wurst, Wolfgang AU - Amadio, Stefano AU - Maggi, Roberto AU - Quattrini, Angelo AU - Consalez, G. Giacomo TI - Hypogonadotropic hypogonadism and peripheral neuropathy in <em>Ebf2</em>-null mice AID - 10.1242/dev.00215 DP - 2003 Jan 15 TA - Development PG - 401--410 VI - 130 IP - 2 4099 - http://dev.biologists.org/content/130/2/401.short 4100 - http://dev.biologists.org/content/130/2/401.full SO - Development2003 Jan 15; 130 AB - Olf/Ebf transcription factors have been implicated in numerous developmental processes, ranging from B-cell development to neuronal differentiation. We describe mice that carry a targeted deletion within the Ebf2 (O/E3) gene. In Ebf2-null mutants, because of defective migration of gonadotropin releasing hormone-synthesizing neurons, formation of the neuroendocrine axis (which is essential for pubertal development) is impaired, leading to secondary hypogonadism. In addition, Ebf2-/- peripheral nerves feature defective axon sorting, hypomyelination, segmental dysmyelination and axonal damage, accompanied by a sharp decrease in motor nerve conduction velocity. Ebf2-null mice reveal a novel genetic cause of hypogonadotropic hypogonadism and peripheral neuropathy in the mouse, disclosing an important role for Ebf2 in neuronal migration and nerve development.