Assessing functional performance in the Mdx mouse model

Journal of Visualized Experiments

Volumn , Issue 85, 2014, Pages

  Aartsma Rus, Annemieke ^a   van Putten, Maaike ^a  

^a LEIDEN UNIVERSITY MEDICAL CENTER   (Netherlands)

Author keywords

Behavior; Duchenne muscular dystrophy; Functional testing; Grip strength; Hanging test grid; Hanging test wire; Issue 85; Mouse model; Neuromuscular disorders; Outcome measures; Rotarod running; Treadmill running

Indexed keywords

EID: 84929384661     PISSN: 1940087X     EISSN: None     Source Type: Journal    
DOI: 10.3791/51303     Document Type: Article

References (21)

1. Blake, D.J., Weir, A., Newey, S.E., Davies, K.E. Function and genetics of dystrophin and dystrophin-related proteins in muscle. Physiol. Rev. 82, 291-329, doi:10.1152/physrev.00028.2001 (2002).
2. Hoffman, E.P., Brown, R.H., Jr., Kunkel, L.M. Dystrophin: the protein product of the Duchenne muscular dystrophy locus. Cell. 51, 919-928, doi:0092-8674(87)90579-4 [pii] (1987).
3. Bushby, K., et al. Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management. Lancet Neurol. 9, 77-93, doi:S1474-4422(09)70271-6 [pii];10.1016/S1474-4422(09)70271-6 (2010).
4. Bulfield, G., Siller, W.G., Wight, P.A., Moore, K.J. X chromosome-linked muscular dystrophy (mdx) in the mouse. Proc. Natl. Acad. Sci. U.S.A. 81, 1189-1192 (1984).
5. Bowles, D.E., et al. Phase 1 gene therapy for Duchenne muscular dystrophy using a translational optimized AAV vector. Mol. Ther. 20, 443-455, doi:mt2011237 [pii];10.1038/mt.2011.237 (2012).
6. Cirak, S., et al. Exon skipping and dystrophin restoration in patients with Duchenne muscular dystrophy after systemic phosphorodiamidate morpholino oligomer treatment: an open-label, phase 2, dose-escalation study. Lancet. 378, 595-605, doi:S0140-6736(11)60756-3 [pii];10.1016/S0140-6736(11)60756-3 (2011).
7. Goemans, N.M., et al. Systemic administration of PRO051 in Duchenne's muscular dystrophy. N. Engl. J. Med. 364, 1513-1522, doi:10.1056/NEJMoa1011367 (2011).
8. Malik, V., et al. Gentamicin-induced readthrough of stop codons in Duchenne muscular dystrophy. Ann. Neurol. 67, 771-780, doi:10.1002/ana.22024 (2010).
9. Skuk, D., et al. First test of a "high-density injection" protocol for myogenic cell transplantation throughout large volumes of muscles in a Duchenne muscular dystrophy patient: eighteen months follow-up. Neuromuscul. Disord. 17, 38-46, doi:S0960-8966(06)00572-4 [pii];10.1016/j.nmd.2006.10.003 (2007).
10. van Putten, M., et al. A 3 months mild functional test regime does not affect disease parameters in young mdx mice. Neuromuscul. Disord. 20, 273-280, doi:S0960-8966(10)00057-X [pii];10.1016/j.nmd.2010.02.004 (2010).
11. De Luca A., et al. Gentamicin treatment in exercised mdx mice: Identification of dystrophin-sensitive pathways and evaluation of efficacy in work-loaded dystrophic muscle. Neurobiol. Dis. 32, 243-253, doi:S0969-9961(08)00157-5 [pii];10.1016/j.nbd.2008.07.009 (2008).
12. Radley-Crabb, H., et al. A single 30min treadmill exercise session is suitable for 'proof-of concept studies' in adult mdx mice: A comparison of the early consequences of two different treadmill protocols. Neuromuscul. Disord. doi:S0960-8966(11)01312-5 [pii];10.1016/j.nmd.2011.07.008 (2011).
13. van Putten, M., et al. The effects of low levels of dystrophin on mouse muscle function and pathology. PLoS. One. 7, e31937, doi:10.1371/journal.pone.0031937;PONE-D-11-16710 [pii] (2012).
14. Willmann, R., et al. Enhancing translation: Guidelines for standard pre-clinical experiments in mdx mice. Neuromuscul. Disord. 1, 43-49, doi:S0960-8966(11)00135-0 [pii];10.1016/j.nmd.2011.04.012 (2011).
15. Connolly, A.M., Keeling, R.M., Mehta, S., Pestronk, A., Sanes, J.R. Three mouse models of muscular dystrophy: the natural history of strength and fatigue in dystrophin-, dystrophin/utrophin-, and laminin alpha2-deficient mice. Neuromuscul. Disord. 11, 703-712, doi:S0960896601002322 [pii] (2001).
16. Rafael, J.A., Nitta, Y., Peters, J., Davies, K.E. Testing of SHIRPA, a mouse phenotypic assessment protocol, on Dmd(mdx) and Dmd(mdx3cv) dystrophin-deficient mice. Mamm. Genome. 11, 725-728, doi:10.1007/s003350010149 [pii] (2000).
17. Chapillon, P., Lalonde, R., Jones, N., Caston, J. Early development of synchronized walking on the rotorod in rats. Effects of training and handling. Behav. Brain Res. 93, 77-81 (1998).
18. Massett, M.P., Berk, B.C. Strain-dependent differences in responses to exercise training in inbred and hybrid mice. Am. J. Physiol. Regul. Integr. Comp. Physiol. 288, R1006-R1013, doi:00476.2004 [pii];10.1152/ajpregu.00476.2004 (2005).
19. Lerman, I., et al. Genetic variability in forced and voluntary endurance exercise performance in seven inbred mouse strains. J. Appl. Physiol. 92, 2245-2255, doi:10.1152/japplphysiol.01045.2001 (2002).
20. Sharp, P.S., Jee, H., Wells, D.J. Physiological characterization of muscle strength with variable levels of dystrophin restoration in mdx mice following local antisense therapy. Mol. Ther. 19, 165-171, doi:mt2010213 [pii];10.1038/mt.2010.213 (2011).
21. Klein, S.M., et al. Noninvasive in vivo assessment of muscle impairment in the mdx mouse model--a comparison of two common wire hanging methods with two different results. J. Neurosci. Methods. 203, 292-297, doi:S0165-0270(11)00601-7 [pii];10.1016/j.jneumeth.2011.10.001 (2012).