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Carboxylases are a class of enzymes that catalyze the addition of a carboxyl group (CO₂ or HCO₃⁻) to target molecules, forming new carbon–carbon bonds. This process is fundamental in various metabolic pathways, including the global carbon cycle and biosynthetic processes in living organisms. Many carboxylases require biotin as a cofactor. Examples include Pyruvate carboxylase and Acetyl-CoA carboxylase. Defects in specific human mitochondrial or cytosolic carboxylases can lead to metabolic disorders due to impaired gluconeogenesis or fatty acid synthesis.
ATP-dependent activation of bicarbonate or CO₂, followed by transfer of the activated CO₂ group to a substrate molecule.
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