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Biotinidase activity is determined colorimetrically by measuring p-aminobenzoate liberation from N-biotinyl-p-aminobenzoate at 546 nm. Activity is determined from a standard curve of p-aminobenzoic acid. Modified Sigma substrate is used.(Wolf B, Grier RE, Allen RJ, et al: Biotinidase deficiency: the enzymatic defect in late-onset carboxylase deficiency. Clin Chim Acta 1983;131:273-281)
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