Data

Correlative Light and Electron Microscopy for the Investigation of Muscle Disease

Monash University
Dr Georg Ramm (Principal investigator) Dr Robert Bryson-Richardson (Principal investigator) Dr Tamar Sztal (Associated with)
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ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Adc&rfr_id=info%3Asid%2FANDS&rft_id=1959.1/1178957&rft.title=Correlative Light and Electron Microscopy for the Investigation of Muscle Disease&rft.identifier=1959.1/1178957&rft.publisher=Monash University&rft.description=In order to investigate the biology of nemaline myopathy, a muscle disease characterised by the formation of nemaline (rod-like) aggregates, we created a zebrafish model.  To generate the model we expressed a disease causing form of ACTA1 (ACTA1D286G) tagged with enhanced green fluorescent protein within the muscle. This allowed the visualisation of disease onset and progression in the living animal. This led to the discovery of how and where the characteristic aggregates form. In order to confirm that the fluorescent aggregates observed in the fish corresponded to those observed in patients using electron microscopy we carried out correlative light and electron microscopy on the zebrafish disease model. This allowed visualisation of both the fluorescent protein and the electron dense aggregates. The raw data from these experiments are provided.&rft.creator=Dr Georg Ramm&rft.creator=Dr Robert Bryson-Richardson&rft.date=2015&rft.relation=https://dx.doi.org/10.1016/B978-0-12-801075-4.00011-2&rft.relation=https://dx.doi.org/10.1007/s00401-015-1430-3 &rft.coverage=AU&rft_rights=Some rights reserved. &rft_rights=This licence lets others distribute, remix, tweak, and build upon your work, even commercially, as long as they credit you for the original creation. https://creativecommons.org/licenses/by/4.0/deed.en&rft_subject=Developmental Genetics (incl. Sex Determination)&rft_subject=BIOLOGICAL SCIENCES&rft_subject=GENETICS&rft_subject=Neurology and Neuromuscular Diseases&rft_subject=MEDICAL AND HEALTH SCIENCES&rft_subject=NEUROSCIENCES&rft.type=dataset&rft.language=English Access the data
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Monash University Victoria 3800 Australia



Full description

In order to investigate the biology of nemaline myopathy, a muscle disease characterised by the formation of nemaline (rod-like) aggregates, we created a zebrafish model.  To generate the model we expressed a disease causing form of ACTA1 (ACTA1D286G) tagged with enhanced green fluorescent protein within the muscle. This allowed the visualisation of disease onset and progression in the living animal. This led to the discovery of how and where the characteristic aggregates form. In order to confirm that the fluorescent aggregates observed in the fish corresponded to those observed in patients using electron microscopy we carried out correlative light and electron microscopy on the zebrafish disease model. This allowed visualisation of both the fluorescent protein and the electron dense aggregates. The raw data from these experiments are provided.

Researchers

Ms Viola Oorschot

Created: 2015-06-17

Data time period: 17 06 2015

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iso31661: AU

Subjects
Biological Sciences | Developmental Genetics (Incl. Sex Determination) | Genetics | Medical and Health Sciences | Neurosciences | Neurology and Neuromuscular Diseases |

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