CoQ10 Deficient Endothelial Cell Culture Model for the Investigation of CoQ10 Blood-Brain Barrier Transport.

ABSTRACT: Primary coenzyme Q10 (CoQ10) deficiency is unique among mitochondrial respiratory chain disorders in that it is potentially treatable if high-dose CoQ10 supplements are given in the early stages of the disease. While supplements improve peripheral abnormalities, neurological symptoms are only partially or temporarily ameliorated. The reasons for this refractory response to CoQ10 supplementation are unclear, however, a contributory factor may be the poor transfer of CoQ10 across the blood-brain barrier (BBB). The aim of this study was to investigate mechanisms of CoQ10 transport across the BBB, using normal and pathophysiological (CoQ10 deficient) cell culture models. The study identifies lipoprotein-associated CoQ10 transcytosis in both directions across the in vitro BBB. Uptake via SR-B1

SUBMITTER: Wainwright L 

PROVIDER: S-EPMC7601674 | biostudies-literature | 2020 Oct

REPOSITORIES: biostudies-literature