Journal
DEVELOPMENT
Volume 130, Issue 2, Pages 401-410Publisher
COMPANY OF BIOLOGISTS LTD
DOI: 10.1242/dev.00215
Keywords
Olf/Ebf genes; Neurogenesis; neural development; neuronal migration; neuroendocrine; GnRH neurons; peripheral nerve; peripheral neuropathy; dysmyclination; homologous recombination; knockout; targeted inactivation; gene targeting; COE2; O/E3
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Funding
- Telethon [GGP030198] Funding Source: Medline
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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 EbJ2 (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.
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