Ketogenic diet plays an important role in treating symptoms of the diseases that affect the central nervous system. It is a high fat, low-carb and moderate protein diet. Glucose is the main energy fuel for the brain, therefore regulation of glucose metabolism is critical for normal brain and entire organism function. After a few days of fasting or a drastic reduction in carbohydrate intake from the diet,lipid metabolism is shifted from normal oxidation to ketogenic metabolism. Due to overproduction of acetyl-CoA and insufficient production of oxaloacetate, liver produce ketone bodies, an alternative energy source for the brain. Ketosis causes changes in energy brain metabolism and can be used for treating severe diseases. Ketogenic diet is typically used by patients with intractable epilepsy, it reduces the frequency of seizures and in some cases it completely prevents them. One of the reasons is increased production of GABA, the main inhibitory neurotransmitter. GLUT1 is the protein that transports glucose across the brain blood barrier. Individuals with GLUT1 deficiency syndrome are treated with ketogenic diet, where ketones serve as an alternative fuel for the brain. A well characterized feature of Alzheimer's disease is progressive decline in cerebral metabolic rate of glucose, ketone bodies improve the effectiveness of glucose metabolism. Amyotrophic lateral sclerosis is a motor neurone disease, where mutation can cause mitochondrial dysfunction and ketogenic diet improves mitochondrial function.
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