title: Assessing functional performance in the mdx mouse model.
abstract: Duchenne muscular dystrophy (DMD) is a severe and progressive muscle wasting disorder for which no cure is available. Nevertheless, several potential pharmaceutical compounds and gene therapy approaches have progressed into clinical trials. With improvement in muscle function being the most important end point in these trials, a lot of emphasis has been placed on setting up reliable, reproducible, and easy to perform functional tests to pre clinically assess muscle function, strength, condition, and coordination in the mdx mouse model for DMD. Both invasive and noninvasive tests are available. Tests that do not exacerbate the disease can be used to determine the natural history of the disease and the effects of therapeutic interventions (e.g. forelimb grip strength test, two different hanging tests using either a wire or a grid and rotarod running). Alternatively, forced treadmill running can be used to enhance disease progression and/or assess protective effects of therapeutic interventions on disease pathology. We here describe how to perform these most commonly used functional tests in a reliable and reproducible manner. Using these protocols based on standard operating procedures enables comparison of data between different laboratories.
authors.affiliation: Department of Human Genetics, Leiden University Medical Center.
authors.forename: Annemieke
authors.initials: A
authors.lastname: Aartsma-Rus
authors.affiliation: Department of Human Genetics, Leiden University Medical Center; M.van_Putten@lumc.nl.
authors.forename: Maaike
authors.initials: M
authors.lastname: van Putten
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D005260:Female; D008297:Male; D051379:Mice; D009043:Motor Activity / Q000502:physiology; D018482:Muscle, Skeletal / Q000503:physiopathology*; D020388:Muscular Dystrophy, Duchenne / Q000175:diagnosis / Q000503:physiopathology*; D010805:Physical Conditioning, Animal; D012420:Running
references.citation: Physiol Rev. 2002 Apr;82(2):291-329
references.pmid: 11917091
references.citation: Ann Neurol. 2010 Jun;67(6):771-80
references.pmid: 20517938
references.citation: Neuromuscul Disord. 2001 Nov;11(8):703-12
references.pmid: 11595512
references.citation: Mol Ther. 2012 Feb;20(2):443-55
references.pmid: 22068425
references.citation: N Engl J Med. 2011 Apr 21;364(16):1513-22
references.pmid: 21428760
references.citation: Neuromuscul Disord. 2007 Jan;17(1):38-46
references.pmid: 17142039
references.citation: Neurobiol Dis. 2008 Nov;32(2):243-53
references.pmid: 18694830
references.citation: Behav Brain Res. 1998 Jun;93(1-2):77-81
references.pmid: 9659989
references.citation: Lancet Neurol. 2010 Jan;9(1):77-93
references.pmid: 19945913
references.citation: Mamm Genome. 2000 Sep;11(9):725-8
references.pmid: 10967129
references.citation: Proc Natl Acad Sci U S A. 1984 Feb;81(4):1189-92
references.pmid: 6583703
references.citation: PLoS One. 2012;7(2):e31937
references.pmid: 22359642
references.citation: Lancet. 2011 Aug 13;378(9791):595-605
references.pmid: 21784508
references.citation: Cell. 1987 Dec 24;51(6):919-28
references.pmid: 3319190
references.citation: Mol Ther. 2011 Jan;19(1):165-71
references.pmid: 20924363
references.citation: Neuromuscul Disord. 2012 Feb;22(2):170-82
references.pmid: 21835619
references.citation: J Neurosci Methods. 2012 Jan 30;203(2):292-7
references.pmid: 22015600
references.citation: Neuromuscul Disord. 2012 Jan;22(1):43-9
references.pmid: 21737275
references.citation: J Appl Physiol (1985). 2002 Jun;92(6):2245-55
references.pmid: 12015333
references.citation: Neuromuscul Disord. 2010 Apr;20(4):273-80
references.pmid: 20307983
references.citation: Am J Physiol Regul Integr Comp Physiol. 2005 Apr;288(4):R1006-13
references.pmid: 15618348