Cerebellar Disorganization Characteristic of Reeler in Scrambler Mutant Mice Despite Presence of Reelin
نویسندگان
چکیده
منابع مشابه
Cerebellar disorganization characteristic of reeler in scrambler mutant mice despite presence of reelin.
Analysis of the molecular basis of neuronal migration in the mammalian CNS relies critically on the discovery and identification of genetic mutations that affect this process. Here, we report the detailed cerebellar phenotype caused by a new autosomal recessive neurological mouse mutation, scrambler (gene symbol scm). The scrambler mutation results in ataxic mice that exhibit several neuroanato...
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The reeler mutation in mice produces an especially well characterized disorder, with systematically abnormal migration of cerebral cortical neurons. The reeler gene encodes a large protein, termed Reelin, that in the cortex is synthesized and secreted exclusively in the Cajal-Retzius neurons of the cortical marginal zone (D'Arcangelo et al., 1995). In reeler mutant mice, loss of Reelin protein ...
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The gene mutated in reeler (reelin) encodes a protein secreted by neurons in the developing brain that controls laminar positioning of migrating cells in the CNS by an unknown mechanism. To investigate Reelin function, we used the nestin promoter to express Reelin ectopically in the ventricular zone and other brain regions in transgenic mice. In the presence of the endogenous protein, ectopic R...
متن کاملDisabled-1 acts downstream of Reelin in a signaling pathway that controls laminar organization in the mammalian brain.
Mutation of either reelin (Reln) or disabled-1 (Dab1) results in widespread abnormalities in laminar structures throughout the brain and ataxia in reeler and scrambler mice. Both exhibit the same neuroanatomical defects, including cerebellar hypoplasia with Purkinje cell ectopia and disruption of neuronal layers in the cerebral cortex and hippocampus. Despite these phenotypic similarities, Reln...
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ژورنال
عنوان ژورنال: The Journal of Neuroscience
سال: 1997
ISSN: 0270-6474,1529-2401
DOI: 10.1523/jneurosci.17-22-08767.1997