Translations:Biotin/25/en

Deficiency as a result of metabolic disorders

Biotinidase deficiency is a deficiency of the enzyme that recycles biotin, the consequence of an inherited genetic mutation. Biotinidase catalyzes the cleavage of biotin from biocytin and biotinyl-peptides (the proteolytic degradation products of each holocarboxylase) and thereby recycles biotin. It is also important in freeing biotin from dietary protein-bound biotin. Neonatal screening for biotinidase deficiency started in the United States in 1984, which as of 2017 was reported as required in more than 30 countries.