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Dietary therapy should focus on high carbohydrates and medium-chain triglycerides (which dont require the carnitine cycle) and a lower intake of long-chain fatty acids
Restoring GSH level is a good tool to deal with the above states but direct oral supplementation with GSH is not very efficient because of its poor absorption and because of its hydrolytic degradation in the digestive system
Excessive hair growth on the face /other areas of the body ( or high male hormones in blood) 3
The majority of cell surface receptors are glycosylated/sialylated, and hyposialylation of muscle glycoproteins is thought to be responsible for muscle deterioration in mouse models of GNE myopathy 17,18,19,20
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