Substrate inhibition of lysosomal hydrolases: α-Galactosidase A and β-glucocerebrosidase
Radosław Kwapiszewski , Barbara Czartoryska , Karina Ziolkowska , Michał Chudy , Artur Dybko , Zbigniew Brzózka
AbstractObjective We have investigated the kinetics of α-galactosidase A and β-glucocerebrosidase deficient in Fabry and Gaucher diseases, respectively. Design and methods We have performed spectrofluorymetric measurements of the activity of enzymes using a derivative of 4-methylumbelliferone as a substrate and a human T-cell line as a source of enzymes. Results We have observed the substrate inhibition effect, which is related to temperature. Conclusions The diagnostic procedures for Fabry and Gaucher diseases used now in laboratory practice neglect temperature-dependent substrate inhibition, which may significantly reduce the sensitivity of enzyme activity determinations.
|Journal series||Clinical Biochemistry, ISSN 0009-9120, (A 30 pkt)|
|Keywords in English||Fabry disease, Gaucher disease, Substrate inhibition, α-Galactosidase A activity, β-glucocerebrosidase activity|
|Publication indicators||= 3; = 3; = 4.0; : 2011 = 0.963; : 2011 = 2.076 (2) - 2011=2.079 (5)|
|Citation count*||4 (2015-05-30)|
* presented citation count is obtained through Internet information analysis and it is close to the number calculated by the Publish or Perish system.