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Usually, they are named after the substrate whose decarboxylation they catalyze, for example pyruvate decarboxylase catalyzes the decarboxylation of pyruvate. Examples [ edit ]
It is a critical co-enzyme of four carboxylases: acetyl CoA carboxylase, which is involved in the synthesis of fatty acids from acetate; pyruvate CoA carboxylase, involved in gluconeogenesis; β-methylcrotonyl CoA carboxylase, involved in the metabolism of leucine; and propionyl CoA carboxylase, which is involved in the metabolism of energy ...
Many of these vitamin K-dependent proteins are involved in coagulation so the function of the encoded enzyme is essential for hemostasis. [5] Most gla domain-containing proteins depend on this carboxylation reaction for posttranslational modification. [6] In humans, the gamma-glutamyl carboxylase enzyme is most highly expressed in the liver.
Control of Acetyl-CoA Carboxylase. The AMP regulated kinase triggers the phosphorylation of the enzyme (thus inactivating it) and the phosphatase enzyme removes the phosphate group. The regulation of mammalian ACC is complex, in order to control two distinct pools of malonyl-CoA that direct either the inhibition of beta oxidation or the ...
Vitamin B complex. Vitamin B 1 (thiamin) Vitamin B 2 (riboflavin) Vitamin B 3 (niacin) Vitamin B 5 (pantothenic acid) Vitamin B 6 group: Pyridoxine; Pyridoxal-5-Phosphate; Pyridoxamine; Vitamin B 7 (biotin) Vitamin B 9 (folate) Vitamin B 12 (cobalamin) Choline; Vitamin A (e.g. retinol (see also - provitamin A carotenoids)) Vitamin C (Ascorbic ...
Genetic disorders such as multiple carboxylase deficiency (MCD) (which includes biotinidase deficiency and holocarboxylase synthetase deficiency) [1] can also lead to inborn or late-onset forms of biotin deficiency. [2] In all cases – dietary, genetic, or otherwise – supplementation with biotin is the primary (and usually only) [3] method ...
Both biotin carboxylase activity as well as the BCCP within the carboxylase complex are highly conserved among this enzyme class. The main source of variation for carboxylases arises from the carboxyltransferase component, as the molecule to which the carboxyl group is transferred (from biotin) dictates the necessary specificity component to ...
The reaction it catalyzes is: pyruvate + HCO − 3 + ATP → oxaloacetate + ADP + P. It is an important anaplerotic reaction that creates oxaloacetate from pyruvate. PC contains a biotin prosthetic group [1] and is typically localized to the mitochondria in eukaryotes with exceptions to some fungal species such as Aspergillus nidulans which have a cytosolic PC.
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