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Dystrophin and utrophin "double knockout" dystrophic mice exhibit a spectrum of degenerative musculoskeletal abnormalities

Isaac, C and Wright, A and Usas, A and Li, H and Tang, Y and Mu, X and Greco, N and Dong, Q and Vo, N and Kang, J and Wang, B and Huard, J (2013) Dystrophin and utrophin "double knockout" dystrophic mice exhibit a spectrum of degenerative musculoskeletal abnormalities. Journal of Orthopaedic Research, 31 (3). 343 - 349. ISSN 0736-0266

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Duchenne muscular dystrophy (DMD) is a degenerative muscle disorder characterized by the lack of dystrophin expression at the sarcolemma of muscle fibers. In addition, DMD patients acquire osteopenia, fragility fractures, and scoliosis indicating that a deficiency in skeletal homeostasis coexists but little is known about the effects of DMD on bone and other connective tissues within the musculoskeletal system. Recent evidence has emerged implicating adult stem cell dysfunction in DMD myopathogenesis. Given the common mesenchymal origin of muscle and bone, we sought to investigate bone and other musculoskeletal tissues in a DMD mouse model. Here, we report that dystrophin-utrophin double knockout (dko) mice exhibit a spectrum of degenerative changes, outside skeletal muscle, in bone, articular cartilage, and intervertebral discs, in addition to reduced lifespan, muscle degeneration, spinal deformity, and cardiomyopathy previously reported. We also report these mice to have a reduced capacity for bone healing and exhibit spontaneous heterotopic ossification in the hind limb muscles. Therefore, we propose the dko mouse as a model for premature musculoskeletal aging and posit that a similar phenomenon may occur in patients with DMD. Copyright © 2012 Orthopaedic Research Society.


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Item Type: Article
Status: Published
CreatorsEmailPitt UsernameORCID
Isaac, C
Wright, A
Usas, A
Tang, Yyit3@pitt.eduYIT3
Mu, X
Greco, N
Dong, Qqid5@pitt.eduQID5
Vo, Nnvv1@pitt.eduNVV1
Kang, Jjdkang@pitt.eduJDKANG
Wang, Bbingwang@pitt.eduBINGWANG
Huard, J
Centers: Other Centers, Institutes, Offices, or Units > Stem Cell Research Center
Date: 1 March 2013
Date Type: Publication
Journal or Publication Title: Journal of Orthopaedic Research
Volume: 31
Number: 3
Page Range: 343 - 349
DOI or Unique Handle: 10.1002/jor.22236
Schools and Programs: School of Medicine > Orthopaedic Surgery
Refereed: Yes
ISSN: 0736-0266
PubMed ID: 23097179
Date Deposited: 31 Mar 2014 15:06
Last Modified: 02 Feb 2019 16:56


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