Mitochondria are subject to unique genetic control by both nuclear DNA and their own
genome, mitochondrial DNA (mtDNA), of which each mitochondrion contains multiple …

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) …

Inherited disorders of oxidative phosphorylation cause the clinically and genetically
heterogeneous diseases known as mitochondrial energy generation disorders, or …

Mitochondria are organelles with vital functions in almost all eukaryotic cells. Often
described as the cellular 'powerhouses' due to their essential role in aerobic oxidative …

Mitochondrial disease presenting in childhood is characterized by clinical, biochemical and
genetic complexity. Some children are affected by canonical syndromes, but the majority …

BACKGROUND: MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and
stroke-like episodes)(OMIM 540000) is the most dominant subtype of mitochondrial …

Mitochondria are critical organelles for endocrine health; steroid hormone biosynthesis
occurs in these organelles and they provide energy in the form of ATP for hormone …

Mitochondrial disorders have emerged as a common cause of inherited disease, but are
traditionally viewed as being difficult to diagnose clinically, and even more difficult to …

Packaging DNA into condensed structures is integral to the transmission of genomes. The
mammalian mitochondrial genome (mtDNA) is a high copy, maternally inherited genome in …

Sequence variation in mitochondrial DNA (mtDNA) was traditionally considered to be
selectively neutral. However, an accumulating body of evidence indicates that this …