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Mitochondrial functions in chronic spinal muscular atrophy.

Authors
  • Gobernado, J M
  • Gosalvez, M
  • Cortina, C
  • Lousa, M
  • Riva, C
  • Gimeno, A
Type
Published Article
Journal
Journal of neurology, neurosurgery, and psychiatry
Publication Date
Jun 01, 1980
Volume
43
Issue
6
Pages
546–549
Identifiers
PMID: 6821580
Source
Medline
License
Unknown

Abstract

We determined the respiration rate, respiratory control and ADP/O ratios, with different substrates in mitochondria isolated from seven patients with chronic spinal muscular atrophy and compared them with normal human muscle. In all cases studied, a severe alteration of the respiratory control with variable derangement of oxidative phosphorylation was found. Similar findings have been described in other neuromuscular disorders including the so-called "mitochondrial myopathy". We believe that this disturbance of mitochondrial function is non specific and only the hypermetabolic syndrome of Luft could be considered biochemically as a "mitochondrial myopathy", a disorder selectively involving mitochondria of skeletal muscle.

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