Abstract
Objectives To study CoQ 10 concentrations in muscle and fibroblast from 6 patients with a CoQ 10 deficiency syndrome. Design and methods CoQ 10 was quantified by HPLC with electrochemical detection. Results Four out of the 6 cases showed muscle CoQ 10 deficiency plus a reduction of mitochondrial respiratory chain enzyme activities. All cases showed decreased CoQ 10 values in fibroblasts when compared with controls. Conclusions Biochemical study of CoQ 10 in both muscle and fibroblasts seems advisable to demonstrate the deficiency in all patients.
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