Muscle phenotype of a rat model of Duchenne muscular dystrophy

Muscle Nerve. 2020 Dec;62(6):757-761. doi: 10.1002/mus.27061. Epub 2020 Sep 22.

Abstract

Introduction: Our aim was to assess key muscle imaging and contractility parameters in the Duchenne muscular dystrophy (DMD) rat model (Dmd-KO rat), which have not yet been characterized sufficiently.

Methods: We performed in-vivo magnetic resonance imaging (MRI) for thigh and leg muscles, and performed hematoxylin and eosin (H&E) staining and in-vivo muscle contractility testing in specific hindlimb muscles.

Results: MRI prior to testing muscle contractility revealed multiple, unevenly distributed focal hyperintensities in the Dmd-KO rat quadriceps and tibialis anterior muscles. H&E staining showed corresponding areas of inflammation and ongoing regeneration. In-vivo contractile testing showed maximal force generated by Dmd-KO muscles was significantly lower, and susceptibility to injury was ~ two-fold greater in the Dmd-KO rats compared to wild-type (WT) rats.

Discussion: Together, the MRI findings, histological findings, and the low strength and high susceptibility to injury in muscles support use of the Dmd-KO rat as an animal model of DMD.

Keywords: Dmd-KO rat; MRI; eccentric injury; muscular dystrophy; skeletal muscle.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Animals, Genetically Modified
  • Disease Models, Animal*
  • Dystrophin / genetics
  • Gene Knockout Techniques
  • Hindlimb
  • Magnetic Resonance Imaging
  • Male
  • Muscle Contraction / genetics
  • Muscle Contraction / physiology*
  • Muscle Strength / genetics
  • Muscle Strength / physiology*
  • Muscle, Skeletal / diagnostic imaging
  • Muscle, Skeletal / pathology
  • Muscle, Skeletal / physiopathology*
  • Muscular Dystrophy, Duchenne / diagnostic imaging
  • Muscular Dystrophy, Duchenne / genetics
  • Muscular Dystrophy, Duchenne / pathology
  • Muscular Dystrophy, Duchenne / physiopathology*
  • Phenotype
  • Quadriceps Muscle / diagnostic imaging
  • Quadriceps Muscle / pathology
  • Quadriceps Muscle / physiopathology
  • Rats*

Substances

  • Dmd protein, rat
  • Dystrophin