Abstract
Biotin is a water-soluble B-complex vitamin, which is an essential coenzyme for four carboxylases enzymes involved in intermediary metabolism. Although the recommended daily intake (RDI) is 30 micrograms, doses as high as 100 times the RDI are widely used for dietary supplementation, therapy of several inherited metabolic diseases (biotin-thiamine responsive basal ganglia disease and biotinidase deficiency) and supportive treatment in patients with disorders of mitochondrial energy metabolism.
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