Effects of Arm Cycling Exercise in Spinal Muscular Atrophy Type II Patients: A Pilot Study

J Child Neurol. 2018 Mar;33(3):209-215. doi: 10.1177/0883073817750500. Epub 2018 Jan 12.

Abstract

Exercise studies in neuromuscular diseases like spinal muscular atrophy (SMA), a devastating disease caused by survival of motor neuron 1 ( SMN1) gene mutations, are drawing attention due to its beneficial effects. In this study, we presented a constructed arm cycling exercise protocol and evaluated the benefits on SMA patients. Five SMA type II patients performed 12 weeks of supervised arm cycling exercise. The physical functions were evaluated together with the SMN2 copy numbers, SMN protein levels, insulin-like growth factor 1(IGF1) and binding protein 3 (IGFBP3) levels. The active cycling distance and duration of patients significantly improved. Significant changes could not have detected either SMN or IGF1 and IGFBP3 levels in response to exercise. The findings demonstrated that the patients tolerated the exercise protocol and gained a benefit from arm cycling but benefits could not be associated with SMN2 copy number, SMN protein level, IGF1, or IGFBP3 levels.

Keywords: IGF1; IGFBP3; SMN protein; SMN2 copy number; arm cycling exercise; spinal muscular atrophy type II.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Arm / physiopathology*
  • Biomarkers / blood
  • Child
  • Child, Preschool
  • Exercise Therapy* / methods
  • Gene Dosage
  • Gene Expression
  • Humans
  • Insulin-Like Growth Factor Binding Protein 3 / blood
  • Insulin-Like Growth Factor I / metabolism
  • Male
  • Pilot Projects
  • Spinal Muscular Atrophies of Childhood / genetics
  • Spinal Muscular Atrophies of Childhood / physiopathology*
  • Spinal Muscular Atrophies of Childhood / therapy*
  • Survival of Motor Neuron 2 Protein / blood
  • Survival of Motor Neuron 2 Protein / genetics
  • Treatment Outcome

Substances

  • Biomarkers
  • IGF1 protein, human
  • IGFBP3 protein, human
  • Insulin-Like Growth Factor Binding Protein 3
  • SMN2 protein, human
  • Survival of Motor Neuron 2 Protein
  • Insulin-Like Growth Factor I