The Effect of Cellular Coenzyme Q₁₀ Deficiency on Lysosomal Acidification

Abstract

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 p 10 (5 µM, 3 days) was able to restore cellular CoQ10 concentration (p p 10 status and the possibility that a deficit in the status of this isoprenoid may result in an impairment of lysosomal acidification.

Keywords

• coenzyme Q₁₀
• lysosome
• mitochondrial
• organelles
• competitive inhibition
• lysosomal pH