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 …

Alternative oxidase ( AOX ) is a non‐mammalian enzyme that can bypass blockade of the
complex III ‐ IV segment of the respiratory chain ( RC ). We crossed a Ciona intestinalis AOX …

Mulibrey nanism is an autosomal recessive prenatal-onset growth disorder characterized by
dysmorphic features, cardiomyopathy, and hepatomegaly. Mutations in TRIM37 encoding a …

Mulibrey nanism is a rare growth disorder of prenatal onset caused by mutations in the TRIM37
gene, which encodes a RING-B-box-coiled-coil protein. The pathogenetic mechanisms …

Myocardial regeneration capacity declines during the first week after birth, and this decline
is linked to adaptation to oxidative metabolism. Utilizing this regenerative window, we …

Mammalian MANF and CDNF proteins are evolutionarily conserved neurotrophic factors that
can protect and repair mammalian dopaminergic neurons in vivo. In Drosophila, the sole …

Mitochondrial disorders are among the most prevalent inborn errors of metabolism but largely
lack treatments and have poor outcomes. High-fat, low-carbohydrate ketogenic diets (KDs) …

Disturbances in the homeostasis of endoplasmic reticulum (ER) referred to as ER stress is
involved in a variety of human diseases. ER stress activates unfolded protein response (UPR)…

Accumulating evidence suggests mitochondria as key modulators of normal and premature
aging, yet whether primary oxidative phosphorylation (OXPHOS) deficiency can cause …