Biotin, sometimes called vitamin H, is an important water-soluble nutrient that aids in the metabolism of fats, carbohydrates, and proteins. It plays a role in the citric acid cycle, and it is also helpful in maintaining a steady blood sugar level. Biotinidase is a hydrolase enzyme needed to free biotin from carboxylases. Without biotinidase activity, the vitamin biotin cannot be used by the body. LOW levels of biotinidase cause biotinidase deficiency, which is an autosomal recessive metabolic disorder in which the body is not able to process the nutrient biotin properly. It may be associated with seizures, developmental delay, eczema, and hearing loss.
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