• Biotinidase cleaves the amide bond of biotinyl-paminobenzoic
acid (B-pABA, an artificial substrate) to
give free biotin and p-aminobenzoic acid (pABA)
• The pABA is diazotized and reacted with a naphthol
reagent to give a purple product which can be
quantitated spectrophotometrically at 546nm
• Biotinidase activity is expressed as nmol of pABA
liberated per minute per ml serum
• Biotinidase deficient cases <0.7 nmol pABA/min/ml
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