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
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|
|Disorders||Biotinidase (BTD) Deficiency|
|Product Brand Name||GSP®|
|Unit Size||1 Kit|
|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|