biostudies-literatureUnknown9(6)Heaton RACoenzyme Q10 (CoQ10) deficiency currently represents the only treatable mitochondrial disorder, however, little is known about how it may affect other organelles. The lysosome has been found to have a large concentration of CoQ10 localised at its membrane; additionally, it has been suggested that it plays a role in the normal acidification of the lysosomal lumen. As a result, in this study we assessed the effect of CoQ10 deficiency on lysosomal acidification. In order to investigate this, a neuronal cell model of CoQ10 deficiency was established via the treatment of SH-SY5Y cells with para-aminobenzoic acid (PABA). This method works through the competitive inhibition of the CoQ10 biosynthetic pathway enzyme, CoQ2. A single 1 mM (5 days) treatment with PABA resulted in a decrease of up to 5Journal of clinical medicineE1923https://www.ebi.ac.uk/biostudies/studies/S-EPMC7355799biostudies-literatureThe Effect of Cellular Coenzyme Q10 Deficiency on Lysosomal Acidification.PMC7355799Heaton RASexton DWHargreaves IHeales SRahman KfalseThe Effect of Cellular Coenzyme Q10 Deficiency on Lysosomal Acidification.Coenzyme Q10 (CoQ10) deficiency currently represents the only treatable mitochondrial disorder, however, little is known about how it may affect other organelles. The lysosome has been found to have a large concentration of CoQ10 localised at its membrane; additionally, it has been suggested that it plays a role in the normal acidification of the lysosomal lumen. As a result, in this study we assessed the effect of CoQ10 deficiency on lysosomal acidification. In order to investigate this, a neuronal cell model of CoQ10 deficiency was established via the treatment of SH-SY5Y cells with para-aminobenzoic acid (PABA). This method works through the competitive inhibition of the CoQ10 biosynthetic pathway enzyme, CoQ2. A single 1 mM (5 days) treatment with PABA resulted in a decrease of up to 52020-01-01T00:00:00Z2020 Jun2025-04-19T18:02:36.665Z2025-04-19T18:02:36.665ZS-EPMC73557993257549410.3390/jcm9061923