Hypogonadotropic hypogonadism and peripheral neuropathy inEbf2-null mice
Hypogonadotropic hypogonadism and peripheral neuropathy inEbf2-null mice
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 theEbf2 (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.
- Vita-Salute San Raffaele University Italy
- Max Planck Society Germany
- University of Genoa Italy
- Helmholtz Zentrum München Germany
- Max Planck Institute of Psychiatry Germany
Male, Hypothalamus, Neural Conduction, Nose, Gonadotropin-Releasing Hormone, Mice, Cell Movement, Basic Helix-Loop-Helix Transcription Factors, Animals, COE2; Dysmyelination; Gene targeting; GnRH neurons; Homologous recombination; Knockout; Neural development; Neuroendocrine; Neurogenesis; Neuronal migration; O/E3; Olf/Ebf genes; Peripheral nerve; Peripheral neuropathy; Targeted inactivation, Olf/Ebf genes; Neurogenesis; Neural development; Neuronal migration; Neuroendocrine; GnRH neurons; Peripheral nerve; Peripheral neuropathy; Dysmyelination; Gene targeting; COE2; O/E3, Mice, Knockout, Neurons, Hypogonadism, Seminiferous Tubules, Sciatic Nerve, DNA-Binding Proteins, Mice, Inbred C57BL, Nasal Mucosa, Gene Targeting, Olf/Ebf genes, Neurogenesis, Neural development, Neuronal migration, Neuroendocrine, GnRH neurons, Peripheral nerve, Peripheral neuropathy, Dysmyelination, Homologous recombination, Knockout, Targeted inactivation, Gene targeting, COE2, O/E3, Female, Transcription Factors
Male, Hypothalamus, Neural Conduction, Nose, Gonadotropin-Releasing Hormone, Mice, Cell Movement, Basic Helix-Loop-Helix Transcription Factors, Animals, COE2; Dysmyelination; Gene targeting; GnRH neurons; Homologous recombination; Knockout; Neural development; Neuroendocrine; Neurogenesis; Neuronal migration; O/E3; Olf/Ebf genes; Peripheral nerve; Peripheral neuropathy; Targeted inactivation, Olf/Ebf genes; Neurogenesis; Neural development; Neuronal migration; Neuroendocrine; GnRH neurons; Peripheral nerve; Peripheral neuropathy; Dysmyelination; Gene targeting; COE2; O/E3, Mice, Knockout, Neurons, Hypogonadism, Seminiferous Tubules, Sciatic Nerve, DNA-Binding Proteins, Mice, Inbred C57BL, Nasal Mucosa, Gene Targeting, Olf/Ebf genes, Neurogenesis, Neural development, Neuronal migration, Neuroendocrine, GnRH neurons, Peripheral nerve, Peripheral neuropathy, Dysmyelination, Homologous recombination, Knockout, Targeted inactivation, Gene targeting, COE2, O/E3, Female, Transcription Factors
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