Importancia del metabolismo de la biotina

摘要: Biotin is a water soluble enzyme cofactor that belongs to the vitamin B complex. In humans, biotin involved in important metabolic pathways such as gluconeogenesis, fatty acid synthesis, and amino catabolism by acting prosthetic group for pyruvate carboxylase, propionyl-CoA beta-methylcrotinyl-CoA acetyl-CoA carboxylase. Carboxylases are synthesized apo-carboxylases without active form produced their covalent binding of epsilon-amino lysine residue apocarboxylases. This reaction catalyzed holo-carboxylase synthetase. The last step degradation carboxylases, cleavage biotinyl moiety from residues, biotinidase results release free biotin, which can be recycled. regulates catabolic carboxylase at posttranscriptional level whereas synthetase regulated transcriptional level. Aside its role regulation gene expression has been implicated induction receptor asialoglycoprotein, glycolytic enzymes egg yolk proteins. deficiency humans extremely rare generally associated with prolonged parenteral nutrition, consumption large quantities avidin, usually raw eggs, severe malnutrition and, inherited disorders. there autosomal recessive disorders metabolism result disruption activity or