HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Argov, Z. Articles by Chance, B. Search for Related Content PubMed PubMed Citation Articles by Argov, Z. Articles by Chance, B.

Bioenergetic heterogeneity of human mitochondrial myopathies

Phosphorus magnetic resonance spectroscopy study

Department of Biochemistry/Biophysics (Dr. Argov, Mr. Maris, and Dr. Chance), University of Pennsylvania, and the Department of Neurology (Dr. Bank), Hospital of the University of Pennsylvania, Philadelphia, PA and the University Health Center (Dr. Peterson), Wayne State University, Detroit, MI.

Twelve adults with mitochondrial myopathies were studied by phosphorus magnetic resonance spectroscopy of muscle. All 12 had abnormal ³¹P-NMR findings; recovery from exercise was abnormal in 11 patients. At rest, the ratio of phosphocreatine to inorganic phosphate was reduced in 10. Exercise transfer characteristics were abnormal in all five patients who could exercise. Exercise-induced intracellular acidosis was subnormal in nine patients. The range of abnormalities indicates biochemical heterogeneity, with two possible groups: (1) primary defects of energy metabolism with marked ³¹P-NMR abnormalities, and (2) secondary, less specific ³¹P-NMR abnormalities.

Address correspondence and reprint requests to Dr. Argov, Department of Biochemistry/Biophysics, University of Pennsylvania, Philadelphia, PA 19104.

Received February 20, 1986. Accepted for publication June 6, 1986.

Supported by National Institute of Health grant NS08075. Dr. Argov is a recipient of a Muscular Dystrophy Association postdoctoral fellowship.

This article has been cited by other articles:

				S.-F. Ko, C.-C. Huang, M.-J. Hsieh, S.-H. Ng, C.-C. Lee, C.-C. Lee, T.-K. Lin, M.-C. Chen, and L. Lee 31P MR Spectroscopic Assessment of Muscle in Patients with Myasthenia Gravis before and after Thymectomy: Initial Experience Radiology, April 1, 2008; 247(1): 162 - 169. [Abstract] [Full Text] [PDF]

				M. Roussel, J. P. Mattei, Y. Le Fur, B. Ghattas, P. J. Cozzone, and D. Bendahan Metabolic determinants of the onset of acidosis in exercising human muscle: a 31P-MRS study J Appl Physiol, March 1, 2003; 94(3): 1145 - 1152. [Abstract] [Full Text] [PDF]

				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]

				M. J. Roef, D.-J. Reijngoud, J. A.L. Jeneson, R. Berger, and K. de Meer Resting oxygen consumption and in vivo ADP are increased in myopathy due to complex I deficiency Neurology, April 9, 2002; 58(7): 1088 - 1093. [Abstract] [Full Text] [PDF]

				M. J Roef, K. de Meer, D.-J. Reijngoud, H. W. Straver, M. de Barse, S. C Kalhan, and R. Berger Triacylglycerol infusion does not improve hyperlactemia in resting patients with mitochondrial myopathy due to complex I deficiency Am. J. Clinical Nutrition, February 1, 2002; 75(2): 228 - 236. [Abstract] [Full Text] [PDF]