Research Paper Volume 11, Issue 22 pp 10338—10355

GBA mutation promotes early mitochondrial dysfunction in 3D neurosphere models

Figure 2. GCase, HEX and β-gal enzymatic activities stabilized from 0 to 6 days of differentiation. GCase activity decreased as expected for the respective genotypes (33% decrease in wt/N370SGBA and 98% decrease in N370SGBA/N370SGBA) when measured at pH 5.4 in the presence of sodium taurocholate. Measurement of GCase at pH 4.5 in the absence of the GCase activator sodium taurocholate yielded similar results, while HEX and β-gal were unaffected. Results are expressed by mean± SEM.