Critical to the evaluation of potential therapeufics (pharmacological, gene, or cell therapies) are sensitive and repeatable physiological assessments of muscle function applied to mouse models of dystrophy. Therefore, we are proposing to continue a research core that performs ex vivo, in situ, and whole animal assessments of muscle integrity and funcfion. This core will not only support the needs of the projects within the center, but will also serve as a nafional resource for performing funcfional evaluation of potenfial therapies for the muscular dystrophies. The Physiological Assessment Core resources are housed in the laboratory space designated to Dr. Barton, the Core Director. Dr. Barton is a muscle physiologist with extensive experience evaluating muscle function in mice. This includes all instrumentation for muscle physiological measurements, specialized equipment for hindlimb suspension experiments, documented free wheel running, and treadmill running, as well as interim storage of samples in both -8OC and liquid nitrogen freezers. We propose to extend our measures to include evaluation of whole animal respiratory funcfion, which will complement ex vivo assessment of the diaphragm. We believe that this Core has provided a previously unmet need in the past 5 years of its existence, and will confinue to be a valuable resource that will enable the enfire muscular dystrophy research community to ufilize these assays to assess the potential benefits of a large number of approaches to the treatment of different forms of muscular dystrophy.

Agency
National Institute of Health (NIH)
Institute
National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Type
Specialized Center--Cooperative Agreements (U54)
Project #
5U54AR052646-09
Application #
8543518
Study Section
Special Emphasis Panel (ZNS1-SRB-S)
Project Start
Project End
Budget Start
2013-08-01
Budget End
2014-07-31
Support Year
9
Fiscal Year
2013
Total Cost
$218,053
Indirect Cost
$59,508
Name
University of Pennsylvania
Department
Type
DUNS #
042250712
City
Philadelphia
State
PA
Country
United States
Zip Code
19104
Posey Jr, Avery D; Swanson, Kaitlin E; Alvarez, Manuel G et al. (2014) EHD1 mediates vesicle trafficking required for normal muscle growth and transverse tubule development. Dev Biol 387:179-90
Villalta, S Armando; Rosenthal, Wendy; Martinez, Leonel et al. (2014) Regulatory T cells suppress muscle inflammation and injury in muscular dystrophy. Sci Transl Med 6:258ra142
Moorwood, Catherine; Barton, Elisabeth R (2014) Caspase-12 ablation preserves muscle function in the mdx mouse. Hum Mol Genet 23:5325-41
Dorn 2nd, Gerald W; McNally, Elizabeth M (2014) Two strikes and you're out: gene-gene mutation interactions in HCM. Circ Res 115:208-10
Swaggart, Kayleigh A; McNally, Elizabeth M (2014) Modifiers of heart and muscle function: where genetics meets physiology. Exp Physiol 99:621-6
Ermolova, N V; Martinez, L; Vetrone, S A et al. (2014) Long-term administration of the TNF blocking drug Remicade (cV1q) to mdx mice reduces skeletal and cardiac muscle fibrosis, but negatively impacts cardiac function. Neuromuscul Disord 24:583-95
Smith, Lucas R; Barton, Elisabeth R (2014) Collagen content does not alter the passive mechanical properties of fibrotic skeletal muscle in mdx mice. Am J Physiol Cell Physiol 306:C889-98
Fahrenbach, John P; Andrade, Jorge; McNally, Elizabeth M (2014) The CO-Regulation Database (CORD): a tool to identify coordinately expressed genes. PLoS One 9:e90408
Puckelwartz, Megan J; Pesce, Lorenzo L; Nelakuditi, Viswateja et al. (2014) Supercomputing for the parallelization of whole genome analysis. Bioinformatics 30:1508-13
Forbes, Sean C; Willcocks, Rebecca J; Triplett, William T et al. (2014) Magnetic resonance imaging and spectroscopy assessment of lower extremity skeletal muscles in boys with Duchenne muscular dystrophy: a multicenter cross sectional study. PLoS One 9:e106435

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