Muscle energy metabolism in human phosphofructokinase deficiency as recorded by 31P nuclear magnetic resonance spectroscopy.
31P 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 muscle tissue adapts to a block in a major bioenergetic pathway.
D000255:Adenosine Triphosphate / Q000378:metabolism; D000328:Adult; D001786:Blood Glucose / Q000378:metabolism; D004734:Energy Metabolism; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D009132:Muscles / Q000201:enzymology / Q000378:metabolism* / Q000503:physiopathology; D010732:Phosphofructokinase-1 / Q000172:deficiency*; D005082:Physical Exertion; D013997:Time Factors
D001786:Blood Glucose; D000255:Adenosine Triphosphate; D010732:Phosphofructokinase-1