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Ketone bodies are produced mainly in the mitochondria of liver cells, and synthesis can occur in response to an unavailability of blood glucose, such as during fasting. [4] Other cells, e.g. human astrocytes, are capable of carrying out ketogenesis, but they are not as effective at doing so. [6] Ketogenesis occurs constantly in a healthy ...
[10] [11] The formation of ketone bodies occurs via ketogenesis in the mitochondrial matrix of liver cells. Fatty acids can be released from adipose tissue by adipokine signaling of high glucagon and epinephrine levels and low insulin levels. High glucagon and low insulin correspond to times of low glucose availability such as fasting. [12]
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.
Ketogenesis occurs primarily in the liver, whereas ketolysis occurs in non-liver cells, especially in the heart, brain, and skeletal muscle. [2] The SCOT enzyme (aka thiophorase) [ 3 ] is required for ketolysis, [ 4 ] and is present in the mitochondria of all mammalian cells except for hepatocytes .
In type 2 diabetes, insulin production is present but is insufficient to meet the body's requirements as a result of end-organ insulin resistance. Usually, these amounts of insulin are sufficient to suppress ketogenesis. If DKA occurs in someone with type 2 diabetes, their condition is called "ketosis-prone type 2 diabetes". [22]
Russell-Silver syndrome can cause pathologic ketotic hypoglycemia. Any genetic or metabolic defect that interferes with the body's ability to maintain glucose homeostasis can trigger pathologic ketotic hypoglycemia, in which episodes of ketotic hypoglycemia persist in children despite increasing age. [2]
Ketoacidosis is a metabolic state caused by uncontrolled production of ketone bodies that cause a metabolic acidosis.While ketosis refers to any elevation of blood ketones, ketoacidosis is a specific pathologic condition that results in changes in blood pH and requires medical attention.
This occurs when blood glucose levels are moderately low such as during sleep, fasting, dieting, [61] pregnancy, and the first 28 days after birth (i.e., the neonatal period); this form of ketogenesis is associated with modest elevations in the blood levels of the ketone bodies and, due to their increased release from adipose tissue, fatty ...