Specific Determination of β-Galactocerebrosidase Activity via Competitive Inhibition of β-Galactosidase

• Published in: Clinical chemistry (Baltimore, Md.) Vol. 55; no. 3; pp. 541 - 548
• Main Authors: Martino, Sabata, Tiribuzi, Roberto, Tortori, Andrea, Conti, Daniele, Visigalli, Ilaria, Lattanzi, Annalisa, Biffi, Alessandra, Gritti, Angela, Orlacchio, Aldo
• Format: Journal Article
• Language: English
• Published: Washington, DC American Association for Clinical Chemistry 01.03.2009
• Subjects: Analytical, structural and metabolic biochemistry; Animals; beta-Galactosidase - antagonists & inhibitors; beta-Galactosidase - metabolism; Biological and medical sciences; Cells, Cultured; Chromatography, Gel; Enzyme Activation - drug effects; Fundamental and applied biological sciences. Psychology; Galactosylceramidase - analysis; Galactosylceramidase - metabolism; Humans; Investigative techniques, diagnostic techniques (general aspects); Medical sciences; Mice; Mice, Inbred C57BL; Silver Nitrate - pharmacology; Tissue Culture Techniques; Glycosylases; Specific activity; Galactosylceramidase; Enzyme; Glycosidases; Clinical biology; Hydrolases; Biochemistry; Molecular biology; Competitive inhibition; β-Galactosidase

Background: The determination of cellular β-galactocerebrosidase activity is an established procedure to diagnose Krabbe disease and monitor the efficacy of gene/stem cell-based therapeutic approaches aimed at restoring defective enzymatic activity in patients or disease models. Current biochemical...