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Item No. 20534

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(+)-Biotin 4-amidobenzoic acid is a biotinidase substrate.1 Upon enzymatic hydrolysis, 4-aminobenzoic acid (PABA; Item No. 18659) is released which can be quantified by colorimetric detection at 540 nm in the presence of N-1-naphthylethylenediamine as a measure of biotinidase activity. (+)-Biotin 4-amidobenzoic acid has been used in the evaluation of biotinidase deficiency, an inborn error of metabolism characterized by ketolactic acidosis, hyperammonemia, and neurological dysfunction.2
WARNING This product is not for human or veterinary use.
1. Biotinyl-
2. Quantitative analytical method for the determination of biotinidase activity in dried blood spot samples. Anal. Chem. 87(20), 10573-10578 (2015).