Tissue triage and freezing for models of skeletal muscle disease

Journal of Visualized Experiments

Volumn , Issue 89, 2014, Pages

  Meng, Hui ^a   Janssen, Paul M L ^b   Grange, Robert W ^c   Yang, Lin ^d   Beggs, Alan H ^e   Swanson, Lindsay C ^e   Cossette, Stacy A ^a,f   Frase, Alison ^f   Childers, Martin K ^f   Granzier, Henk ^g   Gussoni, Emanuela ^e   Lawlor, Michael W ^a  

^a MEDICAL COLLEGE OF WISCONSIN   (United States)

^b OHIO STATE UNIVERSITY   (United States)

^c VIRGINIA POLYTECHNIC INSTITUTE AND STATE UNIVERSITY   (United States)

^d UNIVERSITY OF KENTUCKY   (United States)

^e BOSTON CHILDREN S HOSPITAL   (United States)

^f UNIVERSITY OF WASHINGTON   (United States)

^g UNIVERSITY OF ARIZONA   (United States)

Author keywords

Basic Protocol; Cell culture; Freezing; Functional testing; Isopentane; Issue 89; Muscle; Pathology; Tissue

Indexed keywords

EID: 84904411044     PISSN: 1940087X     EISSN: None     Source Type: Journal    
DOI: 10.3791/51586     Document Type: Article

References (17)

1. Dubowitz, V., & Sewry, C. in Muscle Biopsy: A Practical Approach. Ch. 15, 407-442 Elsevier (2007).
2. Lawlor, M. W. et al. Enzyme replacement therapy rescues weakness and improves muscle pathology in mice with X-linked myotubular myopathy. Hum Mol Genet. 22, 1525-1538, doi:10.1093/hmg/ddt003, (2013).
3. Talmadge, R. J., & Roy, R. R. Electrophoretic separation of rat skeletal muscle myosin heavy-chain isoforms. J Appl Physiol. 75, 2337-2340, (1993).
4. Blau, H. M. et al. Differentiation properties of pure populations of human dystrophic muscle cells. Exp Cell Res. 144, 495-503, (1983).
5. Lawlor, M. W. et al. Myotubularin-deficient myoblasts display increased apoptosis, delayed proliferation, and poor cell engraftment. Am J Pathol. 181, 961-968, doi:10.1016/j.ajpath.2012.05.016, (2012).
6. Pavlath, G. K., & Gussoni, E. Human myoblasts and muscle-derived SP cells. Methods Mol Med. 107, 97-110, (2005).
7. Webster, C., & Blau, H. M. Accelerated age-related decline in replicative life-span of Duchenne muscular dystrophy myoblasts: implications for cell and gene therapy. Somat Cell Mol Genet. 16, 557-565, (1990).
8. Ottenheijm, C. A. et al. Thin filament length dysregulation contributes to muscle weakness in nemaline myopathy patients with nebulin deficiency. Hum Mol Genet. 18, 2359-2369, doi:10.1093/hmg/ddp168, (2009).
9. Witt, C. C. et al. Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo. EMBO J.25, 3843-3855, doi:10.1038/sj.emboj.7601242, (2006).
10. Garton, F. et al. Validation of an automated computational method for skeletal muscle fibre morphometry analysis. Neuromuscul Disord. 20, 540-547, doi:10.1016/j.nmd.2010.06.012, (2010).
11. Kim, Y. J. et al. Fully automated segmentation and morphometrical analysis of muscle fiber images. Cytometry A. 71, 8-15, doi:10.1002/cyto.a.20334, (2007).
12. Lawlor, M. W. et al. Inhibition of activin receptor type IIb increases strength and lifespan in myotubularin-deficient mice. Am J Pathol. 178, 784-793, doi:10.1016/j.ajpath.2010.10.035, (2011).
13. Mula, J. et al. Automated image analysis of skeletal muscle fiber cross-sectional area. J Appl Physiol. 114, 148-155, doi:10.1152/japplphysiol.01022.2012, (2013).
14. Dubowitz, V., & Sewry, C. in Muscle Biopsy: A Practical Approach. Ch. 15, 407-442 Elsevier (2007).
15. Nance, J. R. et al. Congenital myopathies: an update. Curr Neurol Neurosci Rep. 12, 165-174, doi:10.1007/s11910-012-0255-x, (2012).
16. Clarke, N. F. et al. Mutations in TPM3 are a common cause of congenital fiber type disproportion. Ann Neurol. 63, 329-337, doi:10.1002/ana.21308 (2008).
17. Lawlor, M. W. et al. Mutations of tropomyosin 3 (TPM3) are common and associated with type 1 myofiber hypotrophy in congenital fiber type disproportion. Hum Mutat. 31, 176-183, doi:10.1002/humu.21157, (2010).