Abstract:
:To evaluate possible progressive metabolic changes in Duchenne muscular dystrophy, we used 31P nuclear magnetic resonance spectroscopy to measure high-energy phosphate compounds and phosphorylated diesters (PDE) in resting gastrocnemius muscle of 14 Duchenne patients and 10 normal boys. The patients had higher inorganic phosphate (Pi), intracellular pH, and PDE; and lower phosphocreatine (PCr) and PCr/Pi ratio; ATP was not significantly different. The patients showed significant age-related decreases in PCr and PCr/Pi, and increases in Pi and PDE, but ATP did not change. In normal boys, ATP increased with age, but PCr and Pi did not. These studies imply progressive metabolic deterioration in Duchenne dystrophy.
journal_name
Neurologyjournal_title
Neurologyauthors
Younkin DP,Berman P,Sladky J,Chee C,Bank W,Chance Bdoi
10.1212/wnl.37.1.165subject
Has Abstractpub_date
1987-01-01 00:00:00pages
165-9issue
1eissn
0028-3878issn
1526-632Xjournal_volume
37pub_type
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