Specific Determination of {beta}-Galactocerebrosidase Activity via Competitive Inhibition of {beta}-Galactosidase

• Published in: Clinical chemistry (Baltimore, Md.) Vol. 55; no. 3; p. 541
• 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 Am Assoc Clin Chem 01.03.2009; Oxford University Press
• Subjects: Brain; Disease; Enzymatic activity; Gene therapy; Genetic disorders; Health hazards; Mortality; Proteins; Stem cells; Tissues

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 assays for β...