We determined the respiration rate, respiratory control and ADP/O ratios, with different substates 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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CITATION STYLE
Gobernado, J. M., Gosalvez, M., Cortina, C., Lousa, M., Riva, C., & Gimeno, A. (1980). Mitochondrial functions in chronic spinal muscular atrophy. Journal of Neurology Neurosurgery and Psychiatry, 43(6), 546–549. https://doi.org/10.1136/jnnp.43.6.546