Biotinidase deficiency: A metabolic disorder where the body lacks the enzyme biotinidase needed to process the vitamin called biotin (vitamin H) into carboxylase enzymes. More detailed information about the symptoms, causes, and treatments of Biotinidase deficiency is available below.
See full list of 28 symptoms of Biotinidase deficiency
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More information about causes of Biotinidase deficiency:
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Types of Biotinidase deficiency
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The late onset form of MULTIPLE CARBOXYLASE DEFICIENCY (deficiency of the activities of biotin-dependent enzymes propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, and PYRUVATE CARBOXYLASE) due to a defect or deficiency in biotinidase which is essential for recycling BIOTIN. - (Source - Diseases Database)
Biotinidase deficiency is listed as a "rare disease" by the Office of
Rare Diseases (ORD) of the National Institutes of Health
(NIH). This means that Biotinidase deficiency, or a subtype of Biotinidase deficiency,
affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)
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