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| mice with maternal deficiency (m-/p+) for Ube3a resemble human Anagelman Syndrome with motor dysfunction, inducible seizures, and a context-dependent learning deficit  | |
E6-AP knockout mice display an elevated level of Rnf2 and ubiquitinated histone H2A in various tissues, including cerebellar Purkinje neurons, which may have implications to the pathogenesis of Angelman syndrome |
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mice deficient in maternal Ube3a show enetically reversible impairments in both learning and hippocampal long-term potentiation |
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Drosophila model for Angelman Sydrome appear normal externally, but display abnormal locomotive behavior and circadian rhythms, and defective long-term memory. Flies that overexpress Dube3a in the nervous system display locomotion defects |
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dUBE3A-null mutant Drosophila exhibit reduced dendritic branching of sensory neurons in the peripheral nervous system and slowed growth of terminal dendritic fine processes |
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experience-dependent maturation of excitatory cortical circuits is severely impaired and is associated with profound impairments in neocortical plasticity in Angelman syndrome model mice deficient in Ube3a . |
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topotecan unsilenced the paternal Ube3a allele in several regions of the mouse nervous system, including neurons in the hippocampus, neocortex, striatum, cerebellum and spinal cord |