Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Tie JK et al (2016) Characterization of vitamin K-dependent carboxylase mutations that cause bleeding and nonbleeding disorders. Blood 127:1847-1855.

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Enzymes that incorporate C02 as a carboxy group into a substrate. The process catalyzed by a carboxylase is called carboxylation. The process of carboxylation is ATP-dependent. Carboxylases have biotin as a prosthetic group.

General information
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The most important carboxylase is ribulose-1,5-bisphosphate carboxylase, an enzyme that binds carbon dioxide to ribulose-1,5-bisphosphate during photosynthesis (Calvin cycle
)This carboxylation introduces carbon dioxide from water or air into the metabolism of photosynthetic plants.

Other important carboxylases are:

  • Acetyl-CoA-carboxylase, which converts acetyl-coenzyme A to malonyl-coenzyme A (fatty acid synthesis)
  • Pyruvate carboxylase, which converts pyruvate (pyruvic acid) to oxalate (citrateklus)
  • Glutamate carboxylase, which carboxylates glutamic acid to y-carboxyl-glutamic acid (vitamin K cycle)

Carboxylases play an important role in the synthesis of the coagulation factors Factor II, Factor VII, Factor IX, and Factor X as well as Protein S and Protein C. Vitamin K catalyzes the final step in the synthesis of these factors. Here, glutamic acid is carboxylated to y-carboxyl-glutamic acid under the action of a carboxylase, together with CO2 as substrate.

A lack of enzyme activity of pyruvate, propionyl-CoA and 3-methylcrotonyl-CoA carboxylase leads to complex disorders in the breakdown of carbohydrates and the amino acids leucine, isoleucine, valine, threonine and methionine (see below carboxylase defect multiple).

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  1. Narang MA et al (2004) Reduced histone biotinylation in multiple carboxylase deficiency patients: a nuclear role for holocarboxylase synthetase. Hum Mol Genet 13: 15-23
  2. Sweetman L, Nyhan WL (1986) Inheritable biotin-treatable disorders and associated phenomena. Ann Rev Nutr 6: 317-343


Last updated on: 29.10.2020