Muscle metabolic alterations assessed by 31‐phosphorus magnetic resonance spectroscopy in mild becker muscular dystrophy

Although the molecular defect causing Becker muscular dystrophy (BMD) has been identified, the biochemical mechanisms that lead to muscle necrosis remain unclear. Exercise‐related muscle metabolism in 9 mildly affected BMD patients was assessed by muscle 31‐phosphorus magnetic resonance spectroscopy (31P MRS) during an incremental workload. Compared with normal controls, BMD patients showed deregulation of resting pH and intramuscular membrane breakdown. We also observed increased reliance upon anaerobic metabolism during sustained submaximal contraction and maintenance of oxidative function during recovery. Muscle Nerve, 2011

[1]  Susan C. Brown,et al.  Relocalization of neuronal nitric oxide synthase (nNOS) as a marker for complete restoration of the dystrophin associated protein complex in skeletal muscle , 2003, Neuromuscular Disorders.

[2]  P. Cozzone,et al.  New parameters reducing the interindividual variability of metabolic changes during muscle contraction in humans. A (31)P MRS study with physiological and clinical implications. , 2002, Biochimica et biophysica acta.

[3]  D. Graveron-Demilly,et al.  Java-based graphical user interface for the MRUI quantitation package , 2001, Magnetic Resonance Materials in Physics, Biology and Medicine.

[4]  G. D. Thomas,et al.  Functional muscle ischemia in neuronal nitric oxide synthase-deficient skeletal muscle of children with Duchenne muscular dystrophy. , 2000, Proceedings of the National Academy of Sciences of the United States of America.

[5]  D. Arnold,et al.  Insights into muscle diseases gained by phosphorus magnetic resonance spectroscopy , 2000, Muscle & nerve.

[6]  P. Cozzone,et al.  31P magnetic resonance spectroscopy study of phosphocreatine recovery kinetics in skeletal muscle: the issue of intersubject variability. , 2000, Biochimica et biophysica acta.

[7]  C. Juel,et al.  Muscle pH regulation: role of training. , 1998, Acta physiologica Scandinavica.

[8]  A. Blamire,et al.  Correlative MR imaging and 31P-MR spectroscopy study in sarcoglycan deficient limb girdle muscular dystrophy , 1997, Neuromuscular Disorders.

[9]  Vanhamme,et al.  Improved method for accurate and efficient quantification of MRS data with use of prior knowledge , 1997, Journal of magnetic resonance.

[10]  R. Balaban,et al.  Normalized metabolic stress for 31P-MR spectroscopy studies of human skeletal muscle: MVC vs. muscle volume. , 1997, Journal of applied physiology.

[11]  M. Boska,et al.  Adenosine triphosphate production rates, metabolic economy calculations, pH, phosphomonoesters, phosphodiesters, and force output during short‐duration maximal isometric plantar flexion exercises and repeated maximal isometric plantar flexion exercises , 1997, Muscle & nerve.

[12]  J. Pauly,et al.  Short TE phosphorus spectroscopy using a spin‐echo pulse , 1994, Magnetic resonance in medicine.

[13]  S. Frostick,et al.  Cellular energetics of dystrophic muscle , 1993, Journal of the Neurological Sciences.

[14]  P. Zaniol,et al.  31P-NMR spectroscopy of skeletal muscle in Becker dystrophy and DMD/BMD carriers Altered rate of phosphate transport , 1992, Journal of the Neurological Sciences.

[15]  M. Bárány,et al.  Human leg neuromuscular diseases: P-31 MR spectroscopy. , 1989, Radiology.

[16]  B. Chance,et al.  31P NMR studies in Duchenne muscular dystrophy , 1987, Neurology.

[17]  P. Matthews,et al.  metabolic recovery after exercise and the assessment of mitochondrial function in Vivo in human skeletal muscle by means of 31P NMR , 1984, Magnetic resonance in medicine.

[18]  G K Radda,et al.  Nuclear magnetic resonance studies of forearm muscle in Duchenne dystrophy. , 1982, British medical journal.

[19]  P. Vignos,et al.  Maintenance of ambulation in childhood muscular dystrophy. , 1960, Journal of chronic diseases.

[20]  R. Crosbie NO vascular control in Duchenne muscular dystrophy , 2001, Nature Medicine.

[21]  G. Kemp,et al.  Reduced cytosolic acidification during exercise suggests defective glycolytic activity in skeletal muscle of patients with Becker muscular dystrophy , 1999 .

[22]  G. Radda,et al.  Proton efflux in human skeletal muscle during recovery from exercise , 1997, European Journal of Applied Physiology and Occupational Physiology.