Substrate inhibition of lysosomal hydrolases: α-Galactosidase A and β-glucocerebrosidase

Radosław Kwapiszewski , Barbara Czartoryska , Karina Ziolkowska , Michał Chudy , Artur Dybko , Zbigniew Brzózka


Objective 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.
Author Radosław Kwapiszewski (FC / IBC / DM)
Radosław Kwapiszewski,,
- Department Of Microbioanalytics
, Barbara Czartoryska - [Institute of Psychiatry and Neurology, Warszawa]
Barbara Czartoryska,,
, Karina Ziolkowska - [Warsaw University of Technology (PW), MNiSW [80]]
Karina Ziolkowska,,
- Politechnika Warszawska
, Michał Chudy (FC / IBC / DM)
Michał Chudy,,
- Department Of Microbioanalytics
, Artur Dybko (FC / IBC / DM)
Artur Dybko,,
- Department Of Microbioanalytics
, Zbigniew Brzózka (FC / IBC / DM)
Zbigniew Brzózka,,
- Department Of Microbioanalytics
Journal seriesClinical Biochemistry, ISSN 0009-9120, (A 30 pkt)
Issue year2011
Keywords in EnglishFabry disease, Gaucher disease, Substrate inhibition, α-Galactosidase A activity, β-glucocerebrosidase activity
ASJC Classification1308 Clinical Biochemistry; 2700 General Medicine
Languageen angielski
Score (nominal)30
Score sourcejournalList
Publication indicators WoS Citations = 3; Scopus Citations = 3; GS Citations = 4.0; Scopus SNIP (Source Normalised Impact per Paper): 2011 = 0.963; WoS Impact Factor: 2011 = 2.076 (2) - 2011=2.079 (5)
Citation count*4 (2015-05-30)
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* presented citation count is obtained through Internet information analysis and it is close to the number calculated by the Publish or Perish system.
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