Article
Carbohydrate oxidation disorders of skeletal muscle.
Current opinion in clinical nutrition and metabolic care - 1 Nov 2002
Vorgerd Matthias, Zange Jochen
Abstract excerpt
PURPOSE OF REVIEW: The major energy sources for muscle contraction are glycogen, glucose and fatty acids, and defects in their oxidative pathways cause metabolic myopathies. Eleven specific enzyme deficiencies of carbohydrate oxidation affect skeletal muscle alone or in combination with other tissues, such as liver, heart or red blood cells. These hereditary glycogen storage diseases cause two major clinical...
Topics
- Animals
- Disease Models, Animal
- Genetic Therapy
- Genotype
- Glycogen Storage Disease
- Humans
- Magnetic Resonance Spectroscopy
- Muscle, Skeletal
- Oxidation-Reduction
- Phenotype
- Phosphorus Isotopes