A 31P-magnetic resonance spectroscopy and biochemical study of the movbr mouse: Potential model for the mitochondrial encephalomyopathies
✍ Scribed by Irene Tracey; Jeff F. Dunn; George K. Radda
- Publisher
- John Wiley and Sons
- Year
- 1997
- Tongue
- English
- Weight
- 82 KB
- Volume
- 20
- Category
- Article
- ISSN
- 0148-639X
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✦ Synopsis
31
P-magnetic resonance spectroscopy ( 31 P-MRS) provides new biochemical information on mitochondrial disorders affecting brain and muscle. To elucidate the mechanisms of mitochondrial abnormalities, however, animal models are needed. We assessed the mo vbr (mottled viable brindled) mouse for its value in studying (1) energetics of a mitochondrial disorder and (2) 31 P-MRS changes associated with mitochondrial abnormalities in vivo. The maximal activity of succinate-cytochrome c reductase was significantly reduced in mo vbr muscle compared to controls, whereas cytochrome oxidase activity was only reduced in mo vbr brain. 31 P-MRS of mo vbr brain showed an increased pH, but no changes in any metabolite ratios. The phosphocreatine (PCr) recovery rate after exercise was reduced in muscles from mo vbr mice, indicating impairment of oxidative metabolism. We conclude that mo vbr brain and muscle tissue have biochemical abnormalities consistent with mitochondrial impairment. The PCr recovery rate, measured by 31 P-MRS, was sensitive to the muscle abnormality. This strain is best described as having chronic mitochondrial dysfunction.