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The Effect of Cellular Coenzyme Q10 Deficiency on Lysosomal Acidification

Heaton, R, Heales, S, Rahman, K, Sexton, DW and Hargreaves, IP (2020) The Effect of Cellular Coenzyme Q10 Deficiency on Lysosomal Acidification. Journal of Clinical Medicine, 9 (6). ISSN 2077-0383

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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 58% in cellular CoQ10 (p < 0.05). It was found that this resulted in a significant decrease in fluorescence of both the LysoSensor (23%) and LysoTracker (35%) probes used to measure lysosomal pH (p < 0.05). It was found that subsequent treatment with CoQ10 (5 µM, 3 days) was able to restore cellular CoQ10 concentration (p < 0.005), which was associated with an increase in fluorescence from both probes to around 90% of controls (p < 0.05), suggesting a restoration of lysosomal pH. This study provides insights into the association between lysosomal pH and cellular CoQ10 status and the possibility that a deficit in the status of this isoprenoid may result in an impairment of lysosomal acidification.

Item Type: Article
Uncontrolled Keywords: 1103 Clinical Sciences
Subjects: Q Science > QD Chemistry
Divisions: Pharmacy & Biomolecular Sciences
Publisher: MDPI AG
Date Deposited: 19 Jun 2020 15:05
Last Modified: 04 Sep 2021 07:08
URI: https://researchonline.ljmu.ac.uk/id/eprint/13139
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