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Altered mitochondrial oxidative phosphorylation capacity in horses suffering from polysaccharide storage myopathy
Tosi, Irène; Art, Tatiana; Cassart, Dominique et al.
2014In Equine Veterinary Journal, 46 (Suppl 46), p. 9
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Abstract :
[en] Introduction: Exertional myopathies are a common cause of exercise intolerance in the equine athlete, and Polysaccharide Storage Myopathy (PSSM) is a widely described muscular disease. It is characterized by an accumulation of abnormal glycogen in myofibers due to a genetic defect in the skeletal muscle glycogen synthase (GYS1) enzyme. We hypothesized that energy production through oxidative phosphorylation (OXPHOS) in muscular mitochondria might be impaired in type-1 PSSM-affected horses. Methods: Eight horses with a history of exertional rhabdomyolysis were tested for the GYS1 mutation. Muscle biopsies were collected and used for histological analysis and high resolution respirometry (HRR). HRR values from 3 groups of horses (5 PSSM-positive horses, 3 horses with a history of myopathy but PSSM-negative and 16 healthy controls) were compared using a linear mixed model to take into account repeated (2–3 times) measurements made for each horse. Results: In 5/8 horses histology revealed an accumulation of abnormal glycogen in myofibers. These 5 horses also tested positive for the GYS1 mutation. A severe depression of maximal OXPHOS capacitywas observed by HRR in 7/8 horses with exertional rhabdomyolysis, with lower values in PSSM-positive cases (4/5). Conclusions: Our study shows a severely decreased OXPHOS capacity in PSSM-affected horses. PSSM is considered primarily a defect in glycogen synthesis but altered OXPHOS might play a central role in its pathogenesis.
Disciplines :
Veterinary medicine & animal health
Anatomy (cytology, histology, embryology...) & physiology
Author, co-author :
Tosi, Irène ;  Université de Liège - ULiège > Département de sciences fonctionnelles (DSF) > Phys. neuro-muscul., de l'effort - Méd. sport. des animaux
Art, Tatiana ;  Université de Liège - ULiège > Département de sciences fonctionnelles (DSF) > Phys. neuro-muscul., de l'effort - Méd. sport. des animaux
Cassart, Dominique ;  Université de Liège - ULiège > Département de morphologie et pathologie > Département de morphologie et pathologie
Serteyn, Didier  ;  Université de Liège - ULiège > Dép. clinique des animaux de compagnie et des équidés (DCA) > Anesthésiologie gén. et pathologie chirurg. des grds animaux
Votion, Dominique  ;  Université de Liège - ULiège > Scientifiques attachés au Doyen (F MV)
Language :
English
Title :
Altered mitochondrial oxidative phosphorylation capacity in horses suffering from polysaccharide storage myopathy
Publication date :
2014
Event name :
9th International Conference on Equine Exercise Physiology (ICEEP)
Event place :
Chester, United Kingdom
Event date :
2014
Audience :
International
Journal title :
Equine Veterinary Journal
ISSN :
0425-1644
eISSN :
2042-3306
Publisher :
Equine Veterinary Journal Ltd, Newmarket Suffolk, United Kingdom
Volume :
46
Issue :
Suppl 46
Pages :
9
Peer reviewed :
Peer Reviewed verified by ORBi
Available on ORBi :
since 06 April 2015

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