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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 allow visualisation of both the fluorescent protein and the electron dense aggregates. The raw data from these experiments are provided.<br> <br> <b>Related Publications</b><br> <ol> <li>Sztal TE, Zhao M, Williams C, Oorschot V, Parslow AC, Giousoh A, Yuen M, Hall TE, Costin A, Ramm G, Bird PI, Busch-Nentwich EM, Stemple DL, Currie PD, Cooper ST, Laing NG, Nowak KJ, Bryson-Richardson RJ (2015) Zebrafish models for nemaline myopathy reveal a spectrum of nemaline bodies contributing to reduced muscle function. <a href='' target='_blank'>Acta Neuropathol doi: 10.1007/s00401-015-1430-3</a></li> <li>Oorschot VM, Sztal TE, Bryson-Richardson RJ, Ramm G (2014) Immuno correlative light and electron microscopy on Tokuyasu cryosections. <a href='' target='_blank'>Methods Cell Biol 124:241-57</a></li> </ol> <b>Related Organisations</b><br> Monash University<br> <br> <b>Related Grants and Projects</b><br> Determining the pathobiology of human sarcomeric myopathies using zebrafish (funded by National Health and Medical Research Council).<br> <br> <b>Subjects</b><br> Neurology and Neuromuscular Diseases, Developmental Genetics

Monash University
This experiment data is licensed under Creative Commons Attribution 4.0 International (CC BY 4.0).
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