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Ketogenesis is the biochemical process through which organisms produce ketone bodies by breaking down fatty acids and ketogenic amino acids. [1] [2] The process supplies energy to certain organs, particularly the brain, heart and skeletal muscle, under specific scenarios including fasting, caloric restriction, sleep, [3] or others.
Disproven hypotheses include systemic acidosis (high levels of acid in the blood), electrolyte changes and hypoglycaemia (low blood glucose). [21] Although many biochemical changes are known to occur in the brain of a patient on the ketogenic diet, it is not known which of these has an anticonvulsant effect.
Ketosis is a metabolic state characterized by elevated levels of ketone bodies in the blood or urine. Physiological ketosis is a normal response to low glucose availability. In physiological ketosis, ketones in the blood are elevated above baseline levels, but the body's acid–base homeostasis is maintained.
Ketone bodies are water-soluble molecules or compounds that contain the ketone groups produced from fatty acids by the liver (ketogenesis). [1] [2] Ketone bodies are readily transported into tissues outside the liver, where they are converted into acetyl-CoA (acetyl-Coenzyme A) – which then enters the citric acid cycle (Krebs cycle) and is oxidized for energy.
A non-exhaustive list of causes of pathologic ketotic hypoglycemia is listed below: [2] Growth hormone deficiency; Glycogen storage diseases. Glycogen storage disease type IX is a particularly common cause of ketotic hypoglycemia, with the subtype IXa mainly affecting male children [6] Maple syrup urine disease; Prader-Willi syndrome
The action of insulin is counterregulated by glucagon, epinephrine (), norepinephrine (noradrenaline), cortisol, and growth hormone.These counterregulatory hormones—the term is usually used in the plural—raise the level of glucose in the blood by promoting glycogenolysis, gluconeogenesis, ketogenesis, and other catabolic processes. [1]
The brain also uses glucose during starvation, but most of the body's glucose is allocated to the skeletal muscles and red blood cells. The cost of the brain using too much glucose is muscle loss. If the brain and muscles relied entirely on glucose, the body would lose 50% of its nitrogen content in 8–10 days. [13]
Diabetic ketoacidosis (DKA) is a potentially life-threatening complication of diabetes mellitus. [1] Signs and symptoms may include vomiting, abdominal pain, deep gasping breathing, increased urination, weakness, confusion and occasionally loss of consciousness. [1]