Purpose: The purpose of this statement is to review the literature regarding mitochondrial
disease and to provide recommendations for optimal diagnosis and treatment. This …

Leber hereditary optic neuropathy (LHON) and autosomal-dominant optic atrophy (DOA) are
the two most common inherited optic neuropathies in the general population. Both disorders …

Coenzyme Q 10 is a remarkable lipid involved in many cellular processes such as energy
production through the mitochondrial respiratory chain (RC), beta-oxidation of fatty acids …

For a number of years, coenzyme Q10 (CoQ10) was known for its key role in mitochondrial
bioenergetics; later studies demonstrated its presence in other subcellular fractions and in …

Abstract Treatment of mitochondrial respiratory chain (MRC) disorders is extremely difficult,
however, coenzyme Q 10 (CoQ 10) and its synthetic analogues are the only agents which …

Primary Coenzyme Q deficiencies represent a group of rare conditions caused by mutations
in one of the genes required in its biosynthetic pathway at the enzymatic or regulatory level …

Abstract Coenzyme Q10 (CoQ10) is an essential electron carrier in the mitochondrial
respiratory chain and an important antioxidant. Deficiency of CoQ10 is a clinically and …

Chronic kidney disease (CKD) is a severe clinical syndrome with significant socioeconomic
impact worldwide. Orderly energy metabolism is essential for normal kidney function and …

Mitochondrial encephalomyopathy, lactic acidosis, and stroke‐like episodes (MELAS) is a
mitochondrial disease most usually caused by point mutations in tRNA genes encoded by …

Ubiquinone (UQ) is implicated in mitochondrial electron transport, superoxide generation
and as a membrane antioxidant. Here we present a mouse model in which UQ biosynthesis …