Reelin-Related Neurological Disorders and Animal Models

The Reeler mutation was so named because of the alterations in gait that characterize homozygous mice. Several decades after the description of the Reeler phenotype, the mutated protein was discovered and named Reelin (Reln). Reln controls a number of fundamental steps in embryonic and postnatal bra...

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Main Author: Laura Lossi (auth)
Other Authors: Adalberto Merighi (auth), Gabriella D'Arcangelo (auth)
Format: Book Chapter
Published: Frontiers Media SA 2017
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020 |a 978-2-88945-111-1 
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024 7 |a 10.3389/978-2-88945-111-1  |c doi 
041 0 |a English 
042 |a dc 
100 1 |a Laura Lossi  |4 auth 
700 1 |a Adalberto Merighi  |4 auth 
700 1 |a Gabriella D'Arcangelo  |4 auth 
245 1 0 |a Reelin-Related Neurological Disorders and Animal Models 
260 |b Frontiers Media SA  |c 2017 
300 |a 1 electronic resource (179 p.) 
506 0 |a Open Access  |2 star  |f Unrestricted online access 
520 |a The Reeler mutation was so named because of the alterations in gait that characterize homozygous mice. Several decades after the description of the Reeler phenotype, the mutated protein was discovered and named Reelin (Reln). Reln controls a number of fundamental steps in embryonic and postnatal brain development. A prominent embryonic function is the control of radial neuronal migration. As a consequence, homozygous Reeler mutants show disrupted cell layering in cortical brain structures. Reln also promotes postnatal neuronal maturation. Heterozygous mutants exhibit defects in dendrite extension and synapse formation, correlating with behavioral and cognitive deficits that are detectable at adult ages. The Reln-encoding gene is highly conserved between mice and humans. In humans, homozygous RELN mutations cause lissencephaly with cerebellar hypoplasia, a severe neuronal migration disorder that is reminiscent of the Reeler phenotype. In addition, RELN deficiency or dysfunction is also correlated with psychiatric and cognitive disorders, such as schizophrenia, bipolar disorder and autism, as well as some forms of epilepsy and Alzheimer's disease. Despite the wealth of anatomical studies of the Reeler mouse brain, and the molecular dissection of Reln signaling mechanisms, the consequences of Reln deficiency on the development and function of the human brain are not yet completely understood. This Research Topic include reviews that summarize our current knowledge of the molecular aspects of Reln function, original articles that advance our understanding of its expression and function in different brain regions, and reviews that critically assess the potential role of Reln in human psychiatric and cognitive disorders. 
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546 |a English 
653 |a Neurons 
653 |a neuronal migration 
653 |a Schizophrenia 
653 |a Depression 
653 |a Neuronal Death 
653 |a Reeler 
653 |a Synapses 
653 |a autism 
653 |a intracellular pathways 
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