Mitochondrial diseases are a group of genetic disorders that are characterized by defects in
oxidative phosphorylation and caused by mutations in genes in the nuclear DNA (nDNA) …

Metazoans exist only with a continuous and rich supply of chemical energy from oxidative
phosphorylation in mitochondria. The oxidative phosphorylation machinery that mediates …

Most cell functions are carried out by interacting factors, thus underlying the functional
importance of genetic interactions between genes, termed epistasis. Epistasis could be …

Defect of GTPBP3, the human mitochondrial tRNA-modifying enzyme, can lead to Combined
Oxidative Phosphorylation Deficiency 23 (COXPD23). Up to now, about 20 different variants …

Cell death exists in many different forms. Some are accidental, but most of them have some
kind of regulation and are called programmed cell death. Programmed cell death (PCD) is a …

There is extensive evidence from model systems that disrupting associations between co-
adapted mitochondrial and nuclear genotypes can lead to deleterious and even lethal …

Mitochondria play a prominent role in mechanosensory hair cell damage and death.
Although hair cells are thought to be energetically demanding cells, how mitochondria …

Although perhaps best known for their use in developmental studies, over the last couple of
decades, zebrafish have become increasingly popular model organisms for investigating …

Abstract Background Mohr-Tranebjaerg syndrome (MTS) is a rare X-linked recessive
neurodegenerative disorder resulting in early-onset hearing impairment, gradual dystonia …

Of all the human body's sensory systems, the auditory system is perhaps its most intricate.
Hearing loss can result from even modest damage or cell death in the inner ear, and is the …