MUSCLE ENERGY-METABOLISM IN FEMALE DMD BMD CARRIERS - A P-31-MR SPECTROSCOPY STUDY

被引:34
作者
BARBIROLI, B
FUNICELLO, R
FERLINI, A
MONTAGNA, P
ZANIOL, P
机构
[1] UNIV MODENA,IST RADIOL,I-41100 MODENA,ITALY
[2] UNIV MODENA,CATTEDRA ISTOL & EMBRIOL,I-41100 MODENA,ITALY
关键词
DUCHENNE CARRIERS; DMD BMB CARRIERS; MUSCLE P-31-MR SPECTROSCOPY; MUSCLE ENERGY METABOLISM;
D O I
10.1002/mus.880150313
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
P-31 nuclear magnetic resonance spectroscopy (P-31-MRS) was used to investigate the energy metabolism of the gastrocnemius muscle in 16 DMD/BMD female carriers. No significant difference was found with the controls in the resting muscle, while all carriers showed a decreased ability to perform work, and a higher Pi/PCr ratio for comparable relative levels of steady-state work. The rate of postexercise recovery of PCr/Pi ratio was lower in all carriers. The decreased rate of PCr/Pi recovery appears to be mainly due to a decreased rate of Pi recovery. Our findings show abnormal muscle energy metabolism in DMD/BMD female carriers.
引用
收藏
页码:344 / 348
页数:5
相关论文
共 27 条
  • [1] DYSTROPHIN AND THE MEMBRANE HYPOTHESIS OF MUSCULAR-DYSTROPHY
    ARAHATA, K
    SUGITA, H
    [J]. TRENDS IN PHARMACOLOGICAL SCIENCES, 1989, 10 (11) : 437 - 439
  • [2] BIOENERGETIC HETEROGENEITY OF HUMAN MITOCHONDRIAL MYOPATHIES - PHOSPHORUS MAGNETIC-RESONANCE SPECTROSCOPY STUDY
    ARGOV, Z
    BANK, WJ
    MARIS, J
    PETERSON, P
    CHANCE, B
    [J]. NEUROLOGY, 1987, 37 (02) : 257 - 262
  • [3] INVESTIGATION OF HUMAN MITOCHONDRIAL MYOPATHIES BY PHOSPHORUS MAGNETIC-RESONANCE SPECTROSCOPY
    ARNOLD, DL
    TAYLOR, DJ
    RADDA, GK
    [J]. ANNALS OF NEUROLOGY, 1985, 18 (02) : 189 - 196
  • [4] MULTIPLE CONTROLS OF OXIDATIVE-METABOLISM IN LIVING TISSUES AS STUDIED BY PHOSPHORUS MAGNETIC-RESONANCE
    CHANCE, B
    LEIGH, JS
    KENT, J
    MCCULLY, K
    NIOKA, S
    CLARK, BJ
    MARIS, JM
    GRAHAM, T
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1986, 83 (24) : 9458 - 9462
  • [5] CHANCE B, 1985, CIRCULATION, V72, P103
  • [6] PHOSPHORUS NMR-SPECTROSCOPY STUDY OF MUSCULAR ENZYME DEFICIENCIES INVOLVING GLYCOGENOLYSIS AND GLYCOLYSIS
    DUBOC, D
    JEHENSON, P
    DINH, ST
    MARSAC, C
    SYROTA, A
    FARDEAU, M
    [J]. NEUROLOGY, 1987, 37 (04) : 663 - 671
  • [7] EDWARDS RHT, 1982, LANCET, V1, P725
  • [8] P-31 NMR-STUDY OF IMPROVEMENT IN OXIDATIVE-PHOSPHORYLATION BY VITAMIN-K3 AND VITAMIN-C IN A PATIENT WITH A DEFECT IN ELECTRON-TRANSPORT AT COMPLEX-III IN SKELETAL-MUSCLE
    ELEFF, S
    KENNAWAY, NG
    BUIST, NRM
    DARLEYUSMAR, VM
    CAPALDI, RA
    BANK, WJ
    CHANCE, B
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES, 1984, 81 (11): : 3529 - 3533
  • [9] MOLECULAR ANALYSIS OF THE RETT SYNDROME USING CDNA SYNAPSIN-I AS A PROBE
    FERLINI, A
    ANSALONI, L
    NOBILE, C
    FORABOSCO, A
    [J]. BRAIN & DEVELOPMENT, 1990, 12 (01) : 136 - 139
  • [10] GRIFFITHS RD, 1985, MUSCLE NERVE, V8, P760, DOI 10.1002/mus.880080904