Mitochondrial Functions in Chronic Spinal Muscular Atrophy

Overview

Journal J Neurol Neurosurg Psychiatry

Publisher BMJ Publishing Group

Specialties Neurology
Neurosurgery
Psychiatry

Date 1980 Jun 1

PMID 6821580

Citations 3

Authors

J M Gobernado

M Gosalvez

C Cortina

M Lousa

C Riva

A Gimeno

Affiliations

Soon will be listed here.

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.

Citing Articles

Mitochondrial Dysfunction in Spinal Muscular Atrophy.

Zilio E, Piano V, Wirth B Int J Mol Sci. 2022; 23(18).

PMID: 36142791 PMC: 9503857. DOI: 10.3390/ijms231810878.

Altered intracellular Ca2+ homeostasis in nerve terminals of severe spinal muscular atrophy mice.

Ruiz R, Casanas J, Torres-Benito L, Cano R, Tabares L J Neurosci. 2010; 30(3):849-57.

PMID: 20089893 PMC: 6633088. DOI: 10.1523/JNEUROSCI.4496-09.2010.

Mitochondrial myopathies. Clinical, morphological and biochemical aspects.

Sengers R, STADHOUDERS A, Trijbels J Eur J Pediatr. 1984; 141(4):192-207.

PMID: 6329761 DOI: 10.1007/BF00572761.

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