Skip Navigation

This Article
Right arrow Full Text Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Search for citing articles in:
ISI Web of Science (23)
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by Lodi, R.
Right arrow Articles by Taylor, D. J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Lodi, R.
Right arrow Articles by Taylor, D. J.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Brain, Vol. 122, No. 1, 121-130, January 1999
© 1999 Oxford University Press

Reduced cytosolic acidification during exercise suggests defective glycolytic activity in skeletal muscle of patients with Becker muscular dystrophy

An in vivo 31P magnetic resonance spectroscopy study

Raffaele Lodi1, Graham J. Kemp2, Francesco Muntoni3, Campbell H. Thompson1, Caroline Rae1, Jacqueline Taylor3, Peter Styles1 and Doris J. Taylor1

1 MRC Biochemical and Clinical Magnetic Resonance Unit, Oxford University Department of Biochemistry and Oxford Radcliffe Hospital, Oxford, 2 Department of Orthopaedic Surgery, University of Liverpool, Liverpool and 3 Neuromuscular Unit, Department of Paediatrics and Neonatal Medicine, Hammersmith Hospital, London, UK

Correspondence to: Raffaele Lodi, MD, MRC Biochemical and Clinical Magnetic Resonance Unit, Oxford Radcliffe Hospital, Oxford OX3 9DU, UK E-mail: ral{at}bioch.ox.ac.uk

Becker muscular dystrophy is an X-linked disorder due to mutations in the dystrophin gene, resulting in reduced size and/or content of dystrophin. The functional role of this subsarcolemmal protein and the biochemical mechanisms leading to muscle necrosis in Becker muscular dystrophy are still unknown. In particular, the role of a bioenergetic deficit is still controversial. In this study, we used 31P magnetic resonance spectroscopy (31P-MRS) to investigate skeletal muscle mitochondrial and glycolytic ATP production in vivo in 14 Becker muscular dystrophy patients. Skeletal muscle glycogenolytic ATP production, measured during the first minute of exercise, was similar in patients and controls. On the other hand, during later phases of exercise, skeletal muscle in Becker muscular dystrophy patients was less acidic than in controls, the cytosolic pH at the end of exercise being significantly higher in Becker muscular dystrophy patients. The rate of proton efflux from muscle fibres of Becker muscular dystrophy patients was similar to that of controls, pointing to a deficit in glycolytic lactate production as a cause of higher end-exercise cytosolic pH in patients. The maximum rate of mitochondrial ATP production was similar in muscle of Becker muscular dystrophy patients and controls. The results of this in vivo 31P-MRS study are consistent with reduced glucose availability in dystrophin-deficient muscles.

Becker muscular dystrophy; dystrophin; magnetic resonance spectroscopy; energy metabolism; skeletal muscle

PCr = phosphocreatine; Pi = inorganic phosphate; 31P-MRS = phosphorus magnetic resonance spectroscopy


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 Hum Mol GenetHome page
M. Wehling-Henricks, M. Oltmann, C. Rinaldi, K. H. Myung, and J. G. Tidball
Loss of positive allosteric interactions between neuronal nitric oxide synthase and phosphofructokinase contributes to defects in glycolysis and increased fatigability in muscular dystrophy
Hum. Mol. Genet., September 15, 2009; 18(18): 3439 - 3451.
[Abstract] [Full Text] [PDF]

Home page
 CirculationHome page
L. Lee, R. Campbell, M. Scheuermann-Freestone, R. Taylor, P. Gunaruwan, L. Williams, H. Ashrafian, J. Horowitz, A. G. Fraser, K. Clarke, et al.
Metabolic Modulation With Perhexiline in Chronic Heart Failure: A Randomized, Controlled Trial of Short-Term Use of a Novel Treatment
Circulation, November 22, 2005; 112(21): 3280 - 3288.
[Abstract] [Full Text] [PDF]

Home page
 Rheumatology (Oxford)Home page
B. Pfleiderer, J. Lange, K.-D. Loske, and C. Sunderkotter
Metabolic disturbances during short exercises in dermatomyositis revealed by real-time functional 31P magnetic resonance spectroscopy
Rheumatology, June 1, 2004; 43(6): 696 - 703.
[Abstract] [Full Text] [PDF]

Home page
 CirculationHome page
M. Scheuermann-Freestone, P. L. Madsen, D. Manners, A. M. Blamire, R. E. Buckingham, P. Styles, G. K. Radda, S. Neubauer, and K. Clarke
Abnormal Cardiac and Skeletal Muscle Energy Metabolism in Patients With Type 2 Diabetes
Circulation, June 24, 2003; 107(24): 3040 - 3046.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
G. Cea, D. Bendahan, D. Manners, D. Hilton-Jones, R. Lodi, P. Styles, and D. J. Taylor
Reduced oxidative phosphorylation and proton efflux suggest reduced capillary blood supply in skeletal muscle of patients with dermatomyositis and polymyositis: a quantitative 31P-magnetic resonance spectroscopy and MRI study
Brain, July 1, 2002; 125(7): 1635 - 1645.
[Abstract] [Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
J. G. Crilley, E. A. Boehm, B. Rajagopalan, A. M. Blamire, P. Styles, F. Muntoni, D. Hilton-Jones, and K. Clarke
Magnetic resonance spectroscopy evidence of abnormal cardiac energetics in Xp21 muscular dystrophy
J. Am. Coll. Cardiol., November 15, 2000; 36(6): 1953 - 1958.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
J. A. Rafael, E. R. Townsend, S. E. Squire, A. C. Potter, J. S. Chamberlain, and K. E. Davies
Dystrophin and utrophin influence fiber type composition and post-synaptic membrane structure
Hum. Mol. Genet., May 22, 2000; 9(9): 1357 - 1367.
[Abstract] [Full Text] [PDF]


Disclaimer: Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.