Alpha Lipoic Acid Nutritional Support for Genetic Disorders

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Research suggests that alpha lipoic acid could help treat genetic disorders involving metabolism. ^(ii.78)

These include:

Table II.3: Genetic Disorder Treatment with Alpha Lipoic Acid
Disorder	Symptoms and Complications	Alpha Lipoic Acid Dosage or Recommendations
Maple Syrup Urine Disease ^(ii.32)	Ataxia. ^(ii.32, 79) Blindness (occasionally). ^(ii.78) Mental and physical retardation. ^(ii.32, 78)	Combination of supplements of alpha lipoic acid, thiamine, riboflavin, and nicotinamide. ^(ii.78)
Leigh Disease ^(ii.32)	Ataxia. ^(ii.32) Encephalomyelopathy. ^(ii.32) Lactic acidosis. ^(ii.32) Mental retardation. ^(ii.32)	100 mg/day of alpha lipoic acid along with 3000 mg/day of thiamine (other beneficial vitamins include niacin, pantothenate, and riboflavin). ^(ii.32)
Dihydrolipoamide Dehydrogenase Deficiency ^(ii.32)	Severe lactic acidosis. ^(ii.32)	25-50 mg/day of alpha lipoic acid per kg of body weight. ^(ii.32)
Glucose-6-Phosphate Dehydrogenase Deficiency ^(ii.80)	Recessive X-linked hereditary disorder that mostly affects males. ^(ii.81) Mutations in the gene that makes an enzyme for the normal breakdown carbohydrates without building up toxic levels of free radicals. ^(ii.81) Results in hemolytic anemia, which is the rapid destruction of red blood cells. ^(ii.81) Symptoms include paleness, jaundice, fatigue, rapid heart rate, and shortness of breath. ^(ii.81)	In a clinical study, 600 mg/day of alpha lipoic acid increased natural antioxidant levels while reducing indicators of free radical damage in people with and without the disorder. However, this may be especially important since participants with glucose-6-phosphate dehydrogenase deficiency had lower antioxidant levels than those without the disorder. ^(ii.80)
Fanconi Anemia ^(ii.47)	Defective mitochondria in cells, which are associated with increased production of free radicals and strains on natural antioxidant levels. ^(ii.47) Chromosome damage. ^(ii.47) Progressive failure of bone marrow. ^(ii.47) Increased risk of cancer, especially leukemia. ^(ii.47)	Lab studies on cultures from patients with Fanconi Anemia showed that treatment with alpha lipoic acid, with and without N-acetylcysteine, reduces the amount of DNA damage that increases the risk of cancer. The combination of the two antioxidants worked better together than alone. ^(ii.47)

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Loss of coordination. ^(ii.79)

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