Coenzyme Q (CoQ, ubiquinone) is the electron‐carrying lipid in the mitochondrial electron
transport system (ETS). In mammals, it serves as the electron acceptor for nine mitochondrial …

Isolated populations have been valuable for the discovery of rare monogenic diseases and
their causative genetic variants. Finnish disease heritage (FDH) is an example of a group of …

Primary mitochondrial diseases represent some of the most common and severe inherited
metabolic disorders, affecting~ 1 in 4,300 live births. The clinical and molecular diversity …

Background and ethnopharmacological relevance Dioscin and diosgenin derived from
plants of the genus Dioscoreaceae such as D. nipponica and D. panthaica Prain et Burk …

Mitochondrial diseases are extremely heterogeneous genetic conditions characterized by
faulty oxidative phosphorylation (OXPHOS). OXPHOS deficiency can be the result of …

The mitochondrion is a major hub of cellular metabolism and involved directly or indirectly in
almost all biological processes of the cell. In mitochondrial diseases, compromised …

Mitochondria are the powerhouses of the cell. They produce a significant amount of the
energy we need to grow, survive and reproduce. The same system that generates energy in …

Mitochondrial cardiomyopathy (MCM) is characterized by abnormal heart-muscle structure
and function, caused by mutations in the nuclear genome or mitochondrial DNA. The …

Background Septic cardiomyopathy (SCM), a common cardiovascular comorbidity of sepsis,
has emerged among the leading causes of death in patients with sepsis. SCM's …

Energetic insufficiency, excess production of reactive oxygen species (ROS), and aberrant
signaling partially account for the diverse pathology of mitochondrial diseases. Whether …