ABNORMAL HIGH-ENERGY PHOSPHATE-METABOLISM IN HUMAN MUSCLE PHOSPHOFRUCTOKINASE DEFICIENCY

被引:27
作者
BERTOCCI, LA
HALLER, RG
LEWIS, SF
FLECKENSTEIN, JL
NUNNALLY, RL
机构
[1] UNIV TEXAS, SW MED CTR, DEPT NEUROL, DALLAS, TX 75235 USA
[2] UNIV TEXAS, SW MED CTR, DEPT PHYSIOL, DALLAS, TX 75235 USA
[3] VET ADM MED CTR, DALLAS, TX 75216 USA
关键词
NUCLEAR MAGNETIC RESONANCE SPECTROSCOPY; MUSCLE FATIGUE; MUSCLE OXIDATIVE CAPACITY; GLYCOLYSIS; GLYCOGENOLYSIS; PH; ADENOSINE 5'-TRIPHOSPHATE; ADENOSINE 5'-DIPHOSPHATE;
D O I
10.1152/jappl.1991.70.3.1201
中图分类号
Q4 [生理学];
学科分类号
071003 ;
摘要
We studied the pattern of high-energy phosphate metabolism in five patients with phosphofructokinase deficiency (PFKD) and five healthy subjects (HS) during graded rhythmic handgrip performed for 5 min at 17, 33, 50, and 100% of maximal voluntary contraction (MVC). The range of MVC was similar in both groups. Force production was recorded, and intracellular concentrations of phosphorus compounds and pH were measured in the flexor digitorum profundus of the active forearm. At exercise intensities greater-than-or-equal-to 50% MVC, changes in concentrations of high-energy phosphate metabolites were abnormal in PFKD. During maximal effort, [ADP], calculated from the creatine kinase reaction, was 64.3 +/- 13.5 (SE) mu-mol/kg in PFKD vs. 25.7 +/- 4.0 in HS (P < 0.05). Ammonia (NH3), a product of AMP deamination and an index of muscle [AMP], increased approximately twofold more in venous effluent during maximal forearm exercise in PFKD than in HS (P < 0.05). Phosphocreatine concentration was 9.4 +/- 1.3 (SE) mmol/kg in HS and 13.0 +/- 1.7 in PFKD (P < 0.05). Inorganic phosphate concentration was 15.8 +/- 1.4 mmol/kg in HS and 7.4 +/- 0.5 in PFKD (P < 0.05). During strenuous exercise, PFKD patients exhibit an impairment in the rephosphorylation of ADP related to a subnormal oxidative capacity, an absence of glycolysis, and an attenuated breakdown of phosphocreatine.
引用
收藏
页码:1201 / 1207
页数:7
相关论文
共 38 条
  • [1] MUSCLE ENERGY-METABOLISM IN HUMAN PHOSPHOFRUCTOKINASE DEFICIENCY AS RECORDED BY P-31 NUCLEAR-MAGNETIC-RESONANCE SPECTROSCOPY
    ARGOV, Z
    BANK, WJ
    MARIS, J
    LEIGH, JS
    CHANCE, B
    [J]. ANNALS OF NEUROLOGY, 1987, 22 (01) : 46 - 51
  • [2] BLOOD LACTATES AFTER PROLONGED SEVERE EXERCISE
    ASTRAND, PO
    HALLBACK, I
    HEDMAN, R
    SALTIN, B
    [J]. JOURNAL OF APPLIED PHYSIOLOGY, 1963, 18 (03) : 619 - &
  • [3] ATKINSON DE, 1977, CELLULAR ENERGY META
  • [4] CONTROL OF OXIDATIVE-METABOLISM AND OXYGEN DELIVERY IN HUMAN SKELETAL-MUSCLE - A STEADY-STATE ANALYSIS OF THE WORK ENERGY-COST TRANSFER-FUNCTION
    CHANCE, B
    LEIGH, JS
    CLARK, BJ
    MARIS, J
    KENT, J
    NIOKA, S
    SMITH, D
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1985, 82 (24) : 8384 - 8388
  • [5] P-31 NMR-STUDIES OF CONTROL OF MITOCHONDRIAL-FUNCTION IN PHOSPHOFRUCTOKINASE-DEFICIENT HUMAN SKELETAL-MUSCLE
    CHANCE, B
    ELEFF, S
    BANK, W
    LEIGH, JS
    WARNELL, R
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES, 1982, 79 (24): : 7714 - 7718
  • [6] MITOCHONDRIAL REGULATION OF PHOSPHOCREATINE INORGANIC-PHOSPHATE RATIOS IN EXERCISING HUMAN-MUSCLE - A GATED P-31 NMR-STUDY
    CHANCE, B
    ELEFF, S
    LEIGH, JS
    SOKOLOW, D
    SAPEGA, A
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES, 1981, 78 (11): : 6714 - 6718
  • [7] CHASIOTIS D, 1983, ACTA PHYSIOL SCAND, P1
  • [8] THE EFFECTS OF ADP AND PHOSPHATE ON THE CONTRACTION OF MUSCLE-FIBERS
    COOKE, R
    PATE, E
    [J]. BIOPHYSICAL JOURNAL, 1985, 48 (05) : 789 - 798
  • [9] THE INHIBITION OF RABBIT SKELETAL-MUSCLE CONTRACTION BY HYDROGEN-IONS AND PHOSPHATE
    COOKE, R
    FRANKS, K
    LUCIANI, GB
    PATE, E
    [J]. JOURNAL OF PHYSIOLOGY-LONDON, 1988, 395 : 77 - 97
  • [10] MYOFIBRILLAR ACTIVATION FAILURE IN MCARDLES DISEASE
    COOPER, RG
    STOKES, MJ
    EDWARDS, RHT
    [J]. JOURNAL OF THE NEUROLOGICAL SCIENCES, 1989, 93 (01) : 1 - 10