ascorbic-acid and Neuromuscular-Diseases

The interaction between effects of ethanol ingestion and those of infrasound on neuro-muscular fatigue in the animal is abolished by ascorbic acid. The relevant mechanism is in acetaldehyde detoxification.

Topics: Acetaldehyde; Alcoholic Intoxication; Animals; Ascorbic Acid; Fatigue; Humans; Inactivation, Metabolic; Mice; Neuromuscular Diseases; Sound

We determined the respiration, respiratory control, and Pi:O ratios with different substrates in mitochondria isolated from five cases of human neuromuscular disorders (two cases of central core disease, two cases of neuropathy of Dejerine-Sottas, and one case of Kugelberg-Welander's disease) and compared them with normal human muscle. In all the myopathies studied, a severe derangement of the respiratory control with variable derangement of oxidative phosphorylation was found. This supports the idea that a group of neuromyopathies shares the same biochemical lesion as the so-called mitochondrial myopathies, forming with them a group of myopathies which may be related through a similar biochemical lesion of varying degree. Alternatively, disturbance of mitochondrial functions in a number of myopathies could be considered as a non-specific finding.

Topics: Adenosine Diphosphate; Adolescent; Adult; Ascorbic Acid; Child; Female; Glutamates; Humans; Hypertrophy; Infant; Malates; Male; Middle Aged; Mitochondria, Muscle; Motor Neurons; Muscular Atrophy; Muscular Diseases; Myotonic Dystrophy; Neuromuscular Diseases; Oxidative Phosphorylation; Oxidative Phosphorylation Coupling Factors; Oxygen Consumption; Phosphates; Spinal Cord Diseases; Succinates