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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.
It is used to administer the 4:1 ratio classic ketogenic diet in children over one year. The formula is available in both 3:1 and 4:1 ratios, either unflavoured or in an artificially sweetened vanilla flavour and is suitable for tube or oral feeding. [54] Other formula products include KetoVolve [55] and Ketonia. [56]
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.
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 maintain
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.
n/a n/a Ensembl n/a n/a UniProt n a n/a RefSeq (mRNA) n/a n/a RefSeq (protein) n/a n/a Location (UCSC) n/a n/a PubMed search n/a n/a Wikidata View/Edit Human Hydroxymethylglutaryl-CoA lyase HMG-CoA lyase dimer, Human Identifiers EC no. 4.1.3.4 CAS no. 9030-83-5 Databases IntEnz IntEnz view BRENDA BRENDA entry ExPASy NiceZyme view KEGG KEGG entry MetaCyc metabolic pathway PRIAM profile PDB ...
It is a mitochondrial enzyme that catalyzes the second and rate-limiting reaction of ketogenesis, a metabolic pathway that provides lipid-derived energy for various organs during times of carbohydrate deprivation, such as fasting, by addition of a third acetyl group to acetoacetyl-CoA, producing HMG-CoA.
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 .