The Muscle Phenotyping and Imagine Core is designed to enable muscle and non-muscle researchers alike to access a variety of assessment tools for the study of muscle phenotypes. The Core will be useful in 3 main ways. First, the Core will provide a means for investigators to test lead therapeutic compounds in mice to evaluate their effect on muscle pathology. Drugs that delay the onset of disease or reduce muscle pathology will be identified through the small molecule high-throughput screen (Core B) or can be investigator initiated. For these assessments, both traditional (histology, muscle function tests) and non-traditional (optical imaging, MRI) methods will be available for researchers to utilize on a fee for service basis. Second, because most muscular dystrophies involve changes in the dystrophin-glycoprotein complex, biochemical assessments of muscular dystrophy-related proteins will be an additional service offered by this core. Finally, as more and more researchers are making use of transgenic mouse technology, it has become necessary for these individuals to explore muscle phenotypes in their novel knock out and transgenic animals. Since these researchers lack the necessary experience to assess muscle tissue, this Core will provide access to resources, expertise and training necessary to allow them to explore muscle morphology and function in a manner that would not otherwise be available to them.
The Aims of the Core are:
Aim 1 : To enable researchers the opportunity, resources and training to evaluate muscle phenotypes in transgenic mice;
Aim 2 : To enable researchers the opportunity, resources and training to evaluate the efficacy of pharmacological compounds on muscular dystrophy pathogenesis, using quantitative functional and histological outcome measures as well as state-of-the art imaging technologies;
Aim 3 : To enable researchers the opportunity, resources and training to evaluate biochemical outcomes following treatments with pharmacological compounds.

Public Health Relevance

This core facility will be a valuable resource to Center Investigators as well as to the greater dystrophy community by facilitating rapid, pre-clinical testing of compounds. The most promising drugs identified through these combined efforts will be taken to human clinical trials.

Agency
National Institute of Health (NIH)
Institute
National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Type
Center Core Grants (P30)
Project #
5P30AR057230-03
Application #
8286272
Study Section
Special Emphasis Panel (ZAR1)
Project Start
Project End
Budget Start
2011-04-01
Budget End
2012-03-31
Support Year
3
Fiscal Year
2011
Total Cost
$231,162
Indirect Cost
Name
University of California Los Angeles
Department
Type
DUNS #
092530369
City
Los Angeles
State
CA
Country
United States
Zip Code
90095
Wang, Derek W; Mokhonova, Ekaterina I; Kendall, Genevieve C et al. (2018) Repurposing Dantrolene for Long-Term Combination Therapy to Potentiate Antisense-Mediated DMD Exon Skipping in the mdx Mouse. Mol Ther Nucleic Acids 11:180-191
Kramerova, Irina; Torres, Jorge A; Eskin, Ascia et al. (2018) Calpain 3 and CaMKII? signaling are required to induce HSP70 necessary for adaptive muscle growth after atrophy. Hum Mol Genet 27:1642-1653
Aliotta, Eric; Moulin, Kévin; Magrath, Patrick et al. (2018) Quantifying precision in cardiac diffusion tensor imaging with second-order motion-compensated convex optimized diffusion encoding. Magn Reson Med 80:1074-1087
Hicks, Michael R; Hiserodt, Julia; Paras, Katrina et al. (2018) ERBB3 and NGFR mark a distinct skeletal muscle progenitor cell in human development and hPSCs. Nat Cell Biol 20:46-57
Armstrong, Tess; Dregely, Isabel; Stemmer, Alto et al. (2018) Free-breathing liver fat quantification using a multiecho 3D stack-of-radial technique. Magn Reson Med 79:370-382
Wang, Richard T; Barthelemy, Florian; Martin, Ann S et al. (2018) DMD genotype correlations from the Duchenne Registry: Endogenous exon skipping is a factor in prolonged ambulation for individuals with a defined mutation subtype. Hum Mutat 39:1193-1202
Gibbs, Elizabeth M; Crosbie-Watson, Rachelle H (2017) A Simple and Low-cost Assay for Measuring Ambulation in Mouse Models of Muscular Dystrophy. J Vis Exp :
Peter, Angela K; Miller, Gaynor; Capote, Joana et al. (2017) Nanospan, an alternatively spliced isoform of sarcospan, localizes to the sarcoplasmic reticulum in skeletal muscle and is absent in limb girdle muscular dystrophy 2F. Skelet Muscle 7:11
Young, Courtney S; Mokhonova, Ekaterina; Quinonez, Marbella et al. (2017) Creation of a Novel Humanized Dystrophic Mouse Model of Duchenne Muscular Dystrophy and Application of a CRISPR/Cas9 Gene Editing Therapy. J Neuromuscul Dis 4:139-145
Rai, Muhammad Farooq; Duan, Xin; Quirk, James D et al. (2017) Post-Traumatic Osteoarthritis in Mice Following Mechanical Injury to the Synovial Joint. Sci Rep 7:45223

Showing the most recent 10 out of 71 publications