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Biotinidase Deficiency

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Definition: 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.     
See Also Biotin 		Other names Multiple Carboxylase Deficiency, Late Onset; Deficiencies, Biotinidase; Biotinidase Deficiencies; Deficiency, Multiple Carboxylase, Late-Onset; Deficiency, Biotinidase; Multiple Carboxylase Deficiency, Late-Onset; Carboxylase Deficiency, Multiple, Late-Onset
 
SubstanceCAS Registry & nameCategoriesSource
Late-onset multiple carboxylase deficiency  0   *Biotinidase Deficiency.

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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