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Muscle energy metabolism in human phosphofructokinase deficiency as recorded by 31 P nuclear magnetic resonance spectroscopy
Author(s) -
Argov Zohar,
Bank William J.,
Marchis John,
Leigh John S.,
Chance Britton
Publication year - 1987
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410220112
Subject(s) - phosphofructokinase , energy metabolism , nuclear magnetic resonance spectroscopy , nuclear magnetic resonance , glycolysis , metabolism , spectroscopy , medicine , chemistry , biochemistry , physics , quantum mechanics
31 P nuclear magnetic resonance studies of a patient with phosphofructokinase deficiency in muscle provided the following new findings: First, ATP metabolism is disturbed at rest and during exercise. At rest, ATP levels are lower than normal and continue to decline during exercise. Second, exercise kinetics are normal, suggesting a normal mitochondrial fuel supply although glycolysis is blocked. Third, no “phosphate trapping” is observed during prolonged low‐level exercise. Fourth, postexercise recovery is abnormally prolonged by the slow dephosphorylation of sugar phosphates, which has an in vivo half‐life of about nine minutes. These findings demonstrate how musclé tissue adapts to a block in a major bioenergetic pathway.