Coenzyme Q10 deficiency in patients with hereditary hemochromatosis

MARTINEFSKI, MANUELA R.; YAMASATO ,FLORENCIA; DI CARLO, MARÍA B; DARUICH, JORGE; TRIPODI, VALERIA P.

Clinics and Research in Hepatology and Gastroenterology

Elsevier Masson SAS

Año: 2021

2210-7401

Aim: Hereditary hemochromatosis (HH) is a group of inherited disorders that causes a slow andprogressive iron deposition in diverse organs, particularly in the liver. Iron overload inducesoxidative stress and tissue damage. Coenzyme Q10 (CoQ10) is a cofactor in the electrontransport chain of the mitochondria, but it is also a potent endogenous antioxidant. CoQ10interest has recently grown since various studies show that CoQ10 supplementation may provide protective and safe benefits in mitochondrial diseases and oxidative stress disorders. Inthe present study we sought to determine CoQ10 plasma level in patients recently diagnosedwith HH and to correlate it with biochemical, genetic, and histological features of the disease.Methods: Plasma levels of CoQ10, iron, ferritin, transferrin and vitamins (A, C and E), livertests (transaminases, alkaline phosphatase and bilirubin), and histology, as well as three HFEgene mutations (H63D, S654C and C282Y), were assessed in thirty-eight patients (32 males,6 females) newly diagnosed with HH without treatment and in twenty-five age-matched normolipidemic healthy subjects with no HFE gene mutations (22 males, 3 females) and withoutclinical or biochemical signs of iron overload or liver diseases.Results: Patients with HH showed a significant decrease in CoQ10 levels respect to controlsubjects (0.31 ± 0.03 M vs 0.70 ± 0.06 M, p < 0.001,respectively) independently ofthe geneticmutation, cirrhosis, transferrin saturation, ferritin level or markers of hepatic dysfunction.Although a decreasing trend in CoQ10 levels was observed in patients with elevated iron levels, no correlation was found between both parameters in patients with HH. Vitamins C and Alevels showed no changes in HH patients. Vitamin E was significantly decreased in HH patients(21.1 ± 1.3 M vs 29.9 ± 2.5 M, p < 0.001, respectively), but no correlation was observed withCoQ10 levels. Conclusion: The decrease in CoQ10 levels found in HH patients suggests thatCoQ10 supplementation could be a safe intervention strategy complementary to the traditionaltherapy to ameliorate oxidative stress and further tissue damage induced by iron overload.