title: Quantitative phenotyping of Duchenne muscular dystrophy dogs by comprehensive gait analysis and overnight activity monitoring.
abstract: The dystrophin-deficient dog is excellent large animal model for testing novel therapeutic modalities for Duchenne muscular dystrophy (DMD). Despite well-documented descriptions of dystrophic symptoms in these dogs, very few quantitative studies have been performed. Here, we developed a comprehensive set of non-invasive assays to quantify dog gait (stride length and speed), joint angle and limb mobility (for both forelimb and hind limb), and spontaneous activity at night. To validate these assays, we examined three 8-m-old mix-breed dystrophic dogs. We also included three age-matched siblings as the normal control. High-resolution video recorders were used to digitize dog walking and spontaneous movement at night. Stride speed and length were significantly decreased in affected dogs. The mobility of the limb segments (forearm, front foot, lower thigh, rear foot) and the carpus and hock joints was significantly reduced in dystrophic dogs. There was also a significant reduction of the movement in affected dogs during overnight monitoring. In summary, we have established a comprehensive set of outcome measures for clinical phenotyping of DMD dogs. These non-invasive end points would be valuable in monitoring disease progression and therapeutic efficacy in translational studies in the DMD dog model.
authors.affiliation: Department of Molecular Microbiology and Immunology, School of Medicine, The University of Missouri, Columbia, Missouri, United States of America.
authors.forename: Jin-Hong
authors.initials: JH
authors.lastname: Shin
authors.forename: Brian
authors.initials: B
authors.lastname: Greer
authors.forename: Chady H
authors.initials: CH
authors.lastname: Hakim
authors.forename: Zhongna
authors.initials: Z
authors.lastname: Zhou
authors.forename: Yu-chia
authors.initials: YC
authors.lastname: Chung
authors.forename: Ye
authors.initials: Y
authors.lastname: Duan
authors.forename: Zhihai
authors.initials: Z
authors.lastname: He
authors.forename: Dongsheng
authors.initials: D
authors.lastname: Duan
mesh_terms: D000818:Animals; D004285:Dogs; D005121:Extremities / Q000503:physiopathology; D005684:Gait / Q000502:physiology*; D008991:Monitoring, Physiologic / Q000662:veterinary*; D009043:Motor Activity / Q000502:physiology; D009137:Muscular Dystrophy, Animal / Q000503:physiopathology*; D020388:Muscular Dystrophy, Duchenne / Q000503:physiopathology*; D010641:Phenotype; D016059:Range of Motion, Articular / Q000502:physiology
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