{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"omics_type":["Unknown"],"volume":["9(6)"],"submitter":["Heaton RA"],"pubmed_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 5"],"journal":["Journal of clinical medicine"],"pagination":["E1923"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC7355799"],"repository":["biostudies-literature"],"pubmed_title":["The Effect of Cellular Coenzyme Q10 Deficiency on Lysosomal Acidification."],"pmcid":["PMC7355799"],"pubmed_authors":["Heaton RA","Sexton DW","Hargreaves I","Heales S","Rahman K"],"additional_accession":[]},"is_claimable":false,"name":"The Effect of Cellular Coenzyme Q10 Deficiency on Lysosomal Acidification.","description":"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 5","dates":{"release":"2020-01-01T00:00:00Z","publication":"2020 Jun","modification":"2025-04-19T18:02:36.665Z","creation":"2025-04-19T18:02:36.665Z"},"accession":"S-EPMC7355799","cross_references":{"pubmed":["32575494"],"doi":["10.3390/jcm9061923"]}}