Zhang, Ting and Hay, Bruce A. and Guo, Ming (2020) Generation, Analyzing and in-vivo Drug Treatment of Drosophila Models with IBMPFD. Bio-protocol, 10 (10). Art. No. e3621. ISSN 2331-8325. PMCID PMC7842768. doi:10.21769/bioprotoc.3621. https://resolver.caltech.edu/CaltechAUTHORS:20210305-075132983
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Abstract
Missense mutations of p97/cdc48/Valosin-containing protein (VCP) cause inclusion body myopathy, Paget disease with frontotemporal dementia (IBMPFD) and other neurodegenerative diseases. The pathological mechanism of IBMPFD is not clear and there is no treatment. We generated Drosophila models of IBMPFD in adult flight muscle in vivo. Here we describe a variety of assays to characterize disease pathology and dissect disease mechanism, and the consequences of in vivo feeding of VCP inhibitors.
| Item Type: | Article | ||||||||||||||
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| Additional Information: | © 2020 Zhang et al. This article is distributed under the terms of the Creative Commons Attribution License (CC BY 4.0). We are grateful to the generous support from the National Institute of Health (National Institute on Aging), Glenn Foundation for Medical Research, the Natalie R. and Eugene S. Jones Fund in Aging and Neurodegenerative Disease Research, Kenneth Glenn Family Foundation, funds from the UCLA Laurie and Steven Gordon Commitment to Cure Parkinson’s Disease, and Renee and Meyer Luskin Family Fund. We thank Rosaline Young, Mark Dodson, Hansong Deng, and Jina Yun for developing and optimizing the protocol (Clark et al., 2006; Deng et al., 2008; Yun et al., 2014). We have no competing interests. | ||||||||||||||
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| Subject Keywords: | Drosophila, Inclusion body myopathy, Paget disease and frontotemporal dementia (IBMPFD), VCP/p97, Mitochondria, Muscle, Disease models, Inhibitors, Drug treatment | ||||||||||||||
| Issue or Number: | 10 | ||||||||||||||
| PubMed Central ID: | PMC7842768 | ||||||||||||||
| DOI: | 10.21769/bioprotoc.3621 | ||||||||||||||
| Record Number: | CaltechAUTHORS:20210305-075132983 | ||||||||||||||
| Persistent URL: | https://resolver.caltech.edu/CaltechAUTHORS:20210305-075132983 | ||||||||||||||
| Official Citation: | Zhang, T., Hay, B. A. and Guo, M. (2020). Generation, Analyzing and in-vivo Drug Treatment of Drosophila Models with IBMPFD. Bio-protocol 10(10): e3621. DOI: 10.21769/BioProtoc.3621 | ||||||||||||||
| Usage Policy: | No commercial reproduction, distribution, display or performance rights in this work are provided. | ||||||||||||||
| ID Code: | 108322 | ||||||||||||||
| Collection: | CaltechAUTHORS | ||||||||||||||
| Deposited By: | Tony Diaz | ||||||||||||||
| Deposited On: | 08 Mar 2021 20:09 | ||||||||||||||
| Last Modified: | 09 Feb 2022 20:13 |
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