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Brain Structure and Function
Published in: Brain Structure and Function 9/2017

01-12-2017 | Short Communication

Enhanced expression of Pafah1b1 causes over-migration of cerebral cortical neurons into the marginal zone

Authors: Kei-ichi Katayama, Kanehiro Hayashi, Seika Inoue, Kazushige Sakaguchi, Kazunori Nakajima

Published in: Brain Structure and Function | Issue 9/2017

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Abstract

Mutations of PAFAH1B1 cause classical lissencephaly in humans. In addition, duplications and triplications of PAFAH1B1 are found in individuals with intellectual disability and other neurological disorders suggesting that proper brain development is highly sensitive to the PAFAH1B1 dosage. To examine the effect of PAFAH1B1 over-dosage in neural development, especially in migration of neurons and layer formation during cerebral cortical development, we overexpressed Pafah1b1 in migrating neurons in the mouse embryonic cortex using in utero electroporation. Enhanced expression of Pafah1b1 in radially-migrating neurons resulted in their over-migration into the marginal zone. Neurons that invaded the marginal zone were oriented abnormally. Layer distribution of Pafaha1b1-overexpressing neurons shifted more superficially than control neurons. Some of the Pafaha1b1-overexpressing future layer 4 neurons changed their positions to layers 2/3. Furthermore, they also changed their layer marker expression from layer 4 to layers 2/3. These results suggest that overexpression of Pafah1b1 affects the migration of neurons and disrupts layer formation in the developing cerebral cortex, and further support the idea that appropriate dosage of Pafah1b1 is crucial for the proper development of the brain.
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Metadata
Title
Enhanced expression of Pafah1b1 causes over-migration of cerebral cortical neurons into the marginal zone
Authors
Kei-ichi Katayama
Kanehiro Hayashi
Seika Inoue
Kazushige Sakaguchi
Kazunori Nakajima
Publication date
01-12-2017
Publisher
Springer Berlin Heidelberg
Published in
Brain Structure and Function / Issue 9/2017
Print ISSN: 1863-2653
Electronic ISSN: 1863-2661
DOI
https://doi.org/10.1007/s00429-017-1497-9

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