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GlossaryBiotinidase

enzyme recycling biotin, crucial for metabolism and hair health

Biotinidase is an enzyme that plays a crucial role in recycling biotin, a B-vitamin essential for the body's metabolism of fats, carbohydrates, and proteins. Deficiency in biotinidase can lead to biotin deficiency, which may cause symptoms like hair loss, skin rashes, and neurological issues. This enzyme's activity is vital for maintaining adequate biotin levels and overall metabolic health.

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research Phenotypic Variation in Biotinidase Deficiency

184 citations , August 1983 in “The journal of pediatrics/The Journal of pediatrics”

Biotinidase deficiency has various symptoms and can be treated with biotin supplements.

research Biotin And Biotinidase Deficiency

166 citations , November 2008 in “Expert Review of Endocrinology & Metabolism”

Biotin and biotinidase are essential to prevent health issues, and deficiencies require lifelong supplementation.

research Partial Biotinidase Deficiency: Clinical and Biochemical Features

86 citations , January 1990 in “The Journal of Pediatrics”

Children with partial biotinidase deficiency are healthy at birth but may develop symptoms that improve with biotin therapy.

research Low Serum Biotinidase Activity in Children with Valproic Acid Monotherapy

73 citations , October 2001 in “Epilepsia”

Children taking higher doses of valproic acid had lower biotinidase activity, which may lead to biotin deficiency, but biotin supplements could help.

research Biotinidase Deficiency: A Survey of 10 Cases

72 citations , October 1988 in “Archives of Disease in Childhood”

Biotin treatment improves symptoms of biotinidase deficiency, but some neurological damage may be permanent.

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