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Ketogenesis pathway. The three ketone bodies (acetoacetate, acetone, and beta-hydroxy-butyrate) are marked within orange boxes. Ketogenesis is the biochemical process through which organisms produce ketone bodies by breaking down fatty acids and ketogenic amino acids.
The concentration of ketone bodies in blood is maintained around 1 mg/dL. Their excretion in urine is very low and undetectable by routine urine tests (Rothera's test). [18] When the rate of synthesis of ketone bodies exceeds the rate of utilization, their concentration in blood increases; this is known as ketonemia.
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 ...
The ketones are released by the liver into the blood. All cells with mitochondria can take up ketones from the blood and reconvert them into acetyl-CoA, which can then be used as fuel in their citric acid cycles, as no other tissue can divert its oxaloacetate into the gluconeogenic pathway in the way that this can occur in the liver.
When starved, the ketone levels in the shark bodies increases, especially after long-term starvation. Once they are fed, the presence of ketone bodies in the body declines rapidly. The rapid decline is correlated with significant elevations of BHBDH activity, which points towards this enzyme being very important to process ketone bodies. [5]
This deficiency is classified as a disorder ketone body and isoleucine metabolism that can be inherited. [citation needed] Additional mutations include those with the enzymes within pathways related to Acetoacetyl CoA, including Beta-Ketothiolase deficiency and Mitochondrial 3-hydroxy-3-methylglutaryl-CoA Synthase mutation. Mevalonate pathway
A ketogenic amino acid is an amino acid that can be degraded directly into acetyl-CoA, which is the precursor of ketone bodies and myelin, particularly during early childhood, when the developing brain requires high rates of myelin synthesis. [1] This is in contrast to the glucogenic amino acids, which are converted into glucose.
Mutations in this gene are associated with mitochondrial HMG-CoA synthase deficiency (also known as HMGCS2D), affecting ketone body synthesis. [7] Affected patients are unable to perform ketogenesis during starvation and times of higher energy need such as fever and vigorous exercise.