(+)-Biotin 4-Amidobenzoic Acid (sodium salt)
(+)-Biotin 4-amidobenzoic acid is a biotinidase substrate.{32216} 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.{32217}
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