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GSP® Neonatal Biotinidase kit

The GSP Neonatal Biotinidase assay is intended for the quantitative determination of biotinidase activity in blood specimens dried on filter paper as an aid in screening newborns for biotinidase deficiency by using the GSP® instrument.

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The incidence of biotinidase deficiency has been reported as follows: profound biotinidase deficiency (<10% activity) 1 in 112,000, partial deficiency (10%–30% activity) 1 in 129,000, and profound and partial deficiency together 1 in 60,000*. Symptoms include seizure and possible skin disorders, followed by developmental delays, speech problems and possible vision and hearing difficulties. * Kaye, CI. and the Committee on Genetics (2006) Newborn Screening Fact Sheets. Pediatrics 118; 934-963. DOI: 10.1542/peds.2006-1783.

GSP Neonatal Biotinidase - the automated enzymatic/TRF assay

  • First fully automatic assay for biotinidase deficiency screening
  • Improved performance
  • Combination of enzymatic and immunoassay
  • All reagents ready to use (no need for reconstitution)
  • No ethanol precipitation in the assay
  • Pretreated paper for calibrators and controls to avoid lot-to-lot variation caused by the paper

The GSP Neonatal Biotinidase assay combines an enzyme reaction with a solid phase time-resolved immunofluorescence assay. The enzyme reaction is the cleavage by biotinidase of the amide bond in Eu-labeled biotin. The enzyme reaction is stopped by addition of streptavidin which has high affinity for biotin (either Eu-labeled or free biotin). The streptavidin-biotin complexes are captured by the solid phase monoclonal antibody directed against streptavidin. DELFIA® Inducer dissociates the molecules into the solution where the europium fluorescence is measured. The measured fluorescence is inversely proportional to the biotinidase activity of the sample.

All PerkinElmer neonatal products may not be available in all countries.

Disclaimer:  US only


Assay Technology DELFIA and Enzymatic
Calibration Standards Yes
Detection Method Enzymatic
Disorders Biotinidase (BTD) Deficiency
Product Brand Name GSP®
Quantity in a Package Amount 1152.0 Units
Unit Size 1 Kit


Resources, Events & More

Resource Type File Name File Format
Flyer Screening for Biotinidase Deficiency with GSP Neonatal Biotinidase Assay PDF  732 KB
Support Page Newborn screening website Link