Document Detail

Aerobic training as an adjunctive therapy to enzyme replacement in Pompe disease.
MedLine Citation:
PMID:  23041258     Owner:  NLM     Status:  Publisher    
BACKGROUND: Aerobic exercise may be used in conjunction with enzyme replacement therapy (ERT) to attenuate cardiovascular deconditioning, skeletal muscle wasting, and loss of motor function in Pompe disease (glycogen storage disease type II; GSDII), but the effects on lysosomal glycogen content and macroautophagy have not been defined to date. PURPOSE: The main objectives of this study were to determine if acute aerobic exercise enhances 24-h uptake of recombinant human enzyme (rhGAA; Myozyme® [aim 1]) and if endurance training improves disease pathology when combined with ERT [aim 2] in Pompe mice. METHODS: For the first aim in our study, Pompe mutant mice (6(neo)/6(neo)) were grouped into ERT (Myozyme® injection only [40mg/kg]) and ERT+EX (Myozyme® injection followed by 90min treadmill exercise) cohorts, and enzyme uptake was assessed in the heart and quadriceps 24h post injection. For the second aim of our study, mutant mice were randomized into control, endurance-trained, enzyme-treated, or combination therapy groups. Exercised animals underwent 14weeks of progressive treadmill training with or without biweekly Myozyme® injections (40mg/kg) and tissues were harvested 1week post last treatment. RESULTS: Myozyme® uptake (GAA activity) was not improved in ERT+EX over ERT alone at 24-h post injection. Endurance exercise training, with or without ERT, improved aerobic capacity and normalized grip strength, motor function, and lean mass (P<0.05), but did not reduce glycogen content or normalize macroautophagy beyond traditional enzyme replacement therapy. CONCLUSIONS: Endurance training is beneficial as an adjunctive therapy to ERT in Pompe disease, although it works by mechanisms independent of a reduction in glycogen content.
Mats I Nilsson; Imtiaz A Samjoo; Bart P Hettinga; Dwight D Koeberl; Haoyue Zhang; Thomas J Hawke; Aliyah A Nissar; Tahir Ali; Linda Brandt; Mohammad U Ansari; Hassan Hazari; Neha Patel; Jaihoon Amon; Mark A Tarnopolsky
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-9-15
Journal Detail:
Title:  Molecular genetics and metabolism     Volume:  -     ISSN:  1096-7206     ISO Abbreviation:  Mol. Genet. Metab.     Publication Date:  2012 Sep 
Date Detail:
Created Date:  2012-10-8     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9805456     Medline TA:  Mol Genet Metab     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 Elsevier Inc. All rights reserved.
Department of Pediatrics and Medicine, Neuromuscular Clinic, McMaster University, Hamilton, Ontario, Canada.
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