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7 results on '"Davies, Kay E."'

4. Actin Nemaline Myopathy Mouse Reproduces Disease, Suggests Other Actin Disease Phenotypes and Provides Cautionary Note on Muscle Transgene Expression

Author

Ravenscroft, Gianina, primary, Jackaman, Connie, additional, Sewry, Caroline A., additional, McNamara, Elyshia, additional, Squire, Sarah E., additional, Potter, Allyson C., additional, Papadimitriou, John, additional, Griffiths, Lisa M., additional, Bakker, Anthony J., additional, Davies, Kay E., additional, Laing, Nigel G., additional, and Nowak, Kristen J., additional

Published
2011
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5. Daily Treatment with SMTC1100, a Novel Small Molecule Utrophin Upregulator, Dramatically Reduces the Dystrophic Symptoms in the mdx Mouse

Author

Tinsley, Jonathon M., primary, Fairclough, Rebecca J., additional, Storer, Richard, additional, Wilkes, Fraser J., additional, Potter, Allyson C., additional, Squire, Sarah E., additional, Powell, Dave S., additional, Cozzoli, Anna, additional, Capogrosso, Roberta F., additional, Lambert, Adam, additional, Wilson, Francis X., additional, Wren, Stephen P., additional, De Luca, Annamaria, additional, and Davies, Kay E., additional

Published
2011
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6. Utrophin Up-Regulation by an Artificial Transcription Factor in Transgenic Mice

Author

Mattei, Elisabetta, primary, Corbi, Nicoletta, additional, Di Certo, Maria Grazia, additional, Strimpakos, Georgios, additional, Severini, Cinzia, additional, Onori, Annalisa, additional, Desantis, Agata, additional, Libri, Valentina, additional, Buontempo, Serena, additional, Floridi, Aristide, additional, Fanciulli, Maurizio, additional, Baban, Dilair, additional, Davies, Kay E., additional, and Passananti, Claudio, additional

Published
2007
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7. Laf4/Aff3, a Gene Involved in Intellectual Disability, Is Required for Cellular Migration in the Mouse Cerebral Cortex.

Author

Moore, Justin M., Oliver, Peter L., Finelli, Mattéa J., Lee, Sheena, Lickiss, Tom, Molnár, Zoltán, and Davies, Kay E.

Subjects
CEREBRAL cortex, TRANSCRIPTION factors, LEUKEMIA, CELL migration, GENE expression, NERVOUS system
Abstract

Members of the AFF (AF4/FMR2) family of putative transcription factors are involved in infant acute leukaemia and intellectual disability (ID), although very little is known about their transcriptional targets. For example, deletion of human lymphoid nuclear protein related to AF4/AFF member 3 (LAF4/AFF3) is known to cause severe neurodevelopmental defects, and silencing of the gene is also associated with ID at the folate-sensitive fragile site (FSFS) FRA2A; yet the normal function of this gene in the nervous system is unclear. The aim of this study was to further investigate the function of Laf4 in the brain by focusing on its role in the cortex. By manipulating expression levels in organotypic slices, we demonstrate here that Laf4 is required for normal cellular migration in the developing cortex and have subsequently identified Mdga2, an important structural protein in neurodevelopment, as a target of Laf4 transcriptional activity. Furthermore, we show that the migration deficit caused by loss of Laf4 can be partially rescued by Mdga2 over-expression, revealing an important functional relationship between these genes. Our study demonstrates the key transcriptional role of Laf4 during early brain development and reveals a novel function for the gene in the process of cortical cell migration relevant to the haploinsufficiency and silencing observed in human neurodevelopmental disorders. [ABSTRACT FROM AUTHOR]

Published
2014
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