Research Paper Volume 11, Issue 22 pp 10338—10355

GBA mutation promotes early mitochondrial dysfunction in 3D neurosphere models

Figure 3. Confirmation of mitochondrial uncoupling with CCCP in the neurosphere model. Representative blots of OPA1 isoforms. Expected changes from long to short OPA1 isoforms were observed upon uncoupler treatment in all the genotypes, as mitochondria undergo depolarisation/fission following mitochondrial uncoupling (24h, 10 μM CCCP) (A and B). TOM20 levels progressively decrease from 0 to 24h in a control line (C). L-OPA1, long isoform of optic atrophy 1 protein OPA1; S-OPA1, short isoform of OPA1; UT, untreated.