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Your search keyword '"Griffiths, Lisa M."' showing total 6 results
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6 results on '"Griffiths, Lisa M."'

1. Nebulin nemaline myopathy recapitulated in a compound heterozygous mouse model with both a missense and a nonsense mutation in Neb

Author

Laitila, Jenni M., McNamara, Elyshia L., Wingate, Catherine D., Goullee, Hayley, Ross, Jacob A., Taylor, Rhonda L., van der Pijl, Robbert, Griffiths, Lisa M., Harries, Rachel, Ravenscroft, Gianina, Clayton, Joshua S., Sewry, Caroline, Lawlor, Michael W., Ottenheijm, Coen A. C., Bakker, Anthony J., Ochala, Julien, Laing, Nigel G., Wallgren-Pettersson, Carina, Pelin, Katarina, and Nowak, Kristen J.

Published
2020
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2. Rescue of Skeletal Muscle α-Actin-Null Mice by Cardiac (Fetal) α-Actin

Author

Nowak, Kristen J., Ravenscroft, Gianina, Jackaman, Connie, Filipovska, Aleksandra, Davies, Stefan M., Lim, Esther M., Squire, Sarah E., Potter, Allyson C., Baker, Elizabeth, Clément, Sophie, Sewry, Caroline A., Fabian, Victoria, Crawford, Kelly, Lessard, James L., Griffiths, Lisa M., Papadimitriou, John M., Shen, Yun, Morahan, Grant, Bakker, Anthony J., Davies, Kay E., and Laing, Nigel G.

Published
2009
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3. Rescue of skeletal muscle [alpha]-actin--null mice by cardiac (fetal) [alpha]-actin

Author

Nowak, Kristen J., Ravenscroft, Gianina, Jackaman, Connie, Filipovska, Aleksandra, Davies, Stefan M., Lim, Esther M., Squire, Sarah E., Potter, Allyson C., Baker, Elizabeth, Clement, Sophie, Sewry, Caroline A., Fabian, Victoria, Crawford, Kelly, Lessard, James L., Griffiths, Lisa M., Papadimitriou, John M., Shen, Yun, Morahan, Grant, Bakker, Anthony J., Davies, Kay E., and Laing, Nigel G.

Subjects
Muscle proteins -- Analysis, Actin -- Analysis, Biological sciences
Abstract

Skeletal muscle [alpha]-actin (ACTA1) is the major actin in postnatal skeletal muscle. Mutations of ACTA1 cause mostly fatal congenital myopathies. Cardiac [alpha]-actin (ACTC) is the major striated actin in adult heart and fetal skeletal muscle. It is unknown why ACTC and ACTA1 expression switch during development. We investigated whether ACTC can replace ACTA1 in postnatal skeletal muscle. Two ACTC transgenic mouse lines were crossed with Acta1 knockout mice (which all die by 9 d after birth). Offspring resulting from the cross with the high expressing line survive to old age, and their skeletal muscles show no gross pathological features. The mice are not impaired on grip strength, rotarod, or locomotor activity. These findings indicate that ACTC is sufficiently similar to ACTA1 to produce adequate function in postnatal skeletal muscle. This raises the prospect that ACTC reactivation might provide a therapy for ACTA1 diseases. In addition, the mouse model will allow analysis of the precise functional differences between ACTA1 and ACTC.

Published
2009

6. 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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