COMPARISON OF SPECTROPHOTOMETRIC AND FLUORIMETRIC METHODS IN EVALUATION OF BIOTINIDASE DEFICIENCY

İŞERİ ERTEN S. Ö., DİKMEN Z. G., Ulusu N. N.

JOURNAL OF MEDICAL BIOCHEMISTRY, vol.35, no.2, pp.123-129, 2016 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Article
  • Volume: 35 Issue: 2
  • Publication Date: 2016
  • Doi Number: 10.1515/jomb-2016-0004
  • Journal Name: JOURNAL OF MEDICAL BIOCHEMISTRY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.123-129
  • Hacettepe University Affiliated: Yes

Abstract

Background: Biotin, a water-soluble vitamin, is used as a co-factor by enzymes involved in carboxylation reactions. Biotinidase (BTD) catalyzes the recycling of biotin from en do genous and dietary sources. Biotinidase deficiency (BD) is an autosomal recessively inherited disorder of biotin recycling that is associated with neurologic and cutaneous consequences when untreated. The aim of the study was to compare the results of spectrophotometric and fluorimetric methods, as well as to evaluate the advantages and disadvantages of both methods in current research practices.