Endurance exercise leads to beneficial molecular and physiological effects in a mouse model of myotonic dystrophy type 1

Muscle Nerve. 2019 Dec;60(6):779-789. doi: 10.1002/mus.26709. Epub 2019 Oct 23.

Abstract

Introduction: Myotonic dystrophy type 1 (DM1) is a multisystemic disease caused by expansion of a CTG repeat in the 3' UTR of the Dystrophia Myotonica-Protein Kinase (DMPK) gene. While multiple organs are affected, more than half of mortality is due to muscle wasting.

Methods: It is unclear whether endurance exercise provides beneficial effects in DM1. Here, we show that a 10-week treadmill endurance exercise program leads to beneficial effects in the HSALR mouse model of DM1.

Results: Animals that performed treadmill training displayed reduced CUGexp RNA levels, improved splicing abnormalities, an increase in skeletal muscle weight and improved endurance capacity.

Discussion: These results indicate that endurance exercise does not have adverse effects in HSALR animals and contributes to beneficial molecular and physiological outcomes.

Keywords: endurance exercise; mouse model; myotonic dystrophy type I; skeletal muscle; splicing; treadmill.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Actins / genetics
  • Adipose Tissue
  • Alternative Splicing
  • Animals
  • Body Composition
  • Bone Density
  • Disease Models, Animal
  • Endurance Training / methods*
  • Gene Expression
  • Humans
  • Mice
  • Mice, Transgenic
  • Muscle, Skeletal / metabolism*
  • Muscle, Skeletal / pathology
  • Muscle, Skeletal / physiopathology
  • Myotonic Dystrophy / metabolism*
  • Myotonic Dystrophy / pathology
  • Myotonic Dystrophy / physiopathology
  • Organ Size
  • Physical Conditioning, Animal / methods*
  • Physical Endurance / physiology*
  • Reverse Transcriptase Polymerase Chain Reaction
  • Trinucleotide Repeat Expansion

Substances

  • ACTA1 protein, human
  • Actins