Journal article
Dystrophin and utrophin "double knockout" dystrophic mice exhibit a spectrum of degenerative musculoskeletal abnormalities
Journal of orthopaedic research, Vol.31(3), pp.343-349
03/2013
DOI: 10.1002/jor.22236
PMCID: PMC4108902
PMID: 23097179
Abstract
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.
Details
- Title: Subtitle
- Dystrophin and utrophin "double knockout" dystrophic mice exhibit a spectrum of degenerative musculoskeletal abnormalities
- Creators
- Christian Isaac - Stem Cell Research Center, Bridgeside Point II, 450 Technology Dr, Suite 206, Pittsburgh, Pennsylvania 15219, USA.Adam Wright - University of PittsburghArvydas Usas - Stem Cell Research Center, Bridgeside Point II, 450 Technology Dr, Suite 206, Pittsburgh, Pennsylvania 15219Hongshuai LiYing Tang - Stem Cell Research Center, Bridgeside Point II, 450 Technology Dr, Suite 206, Pittsburgh, Pennsylvania 15219Xiaodong Mu - Stem Cell Research Center, Bridgeside Point II, 450 Technology Dr, Suite 206, Pittsburgh, Pennsylvania 15219Nicholas Greco - University of PittsburghQing Dong - University of PittsburghNam Vo - University of PittsburghJames Kang - University of PittsburghBing Wang - University of PittsburghJohnny Huard - University of Pittsburgh
- Resource Type
- Journal article
- Publication Details
- Journal of orthopaedic research, Vol.31(3), pp.343-349
- Publisher
- Wiley Subscription Services, Inc., A Wiley Company
- DOI
- 10.1002/jor.22236
- PMID
- 23097179
- PMCID
- PMC4108902
- ISSN
- 0736-0266
- eISSN
- 1554-527X
- Number of pages
- 7
- Language
- English
- Date published
- 03/2013
- Academic Unit
- Orthopedics and Rehabilitation; Radiation Oncology
- Record Identifier
- 9984304699702771
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