The maintenance of mitochondrial DNA (mtDNA) depends on a number of nuclear gene-
encoded proteins including a battery of enzymes forming the replisome needed to …

Mitochondria are complex organelles that harbour their own genome. Mitochondrial DNA
(mtDNA) exists in the form of a circular double-stranded DNA molecule that must be …

Mitophagy is a fundamental quality control mechanism of mitochondria. Its regulatory
mechanisms and pathological implications remain poorly understood. Here, via a …

Although mitochondrial dysfunction is the known cause of primary mitochondrial disease,
mitochondrial dysfunction is often difficult to measure and prove, especially when biopsies of …

Mutations in the genes encoding Leucine Rich Repeat (LRR) containing proteins are
associated with over sixty human diseases; these include high myopia, mitochondrial …

Mitochondria do not exist as individual entities in the cell—conversely, they constitute an
interconnected community governed by the constant and opposite process of fission and …

The dynamic nature of mitochondria, which can fuse, divide and move throughout the cell,
allows these critical organelles to adapt their function in response to cellular demands, and …

F‐box and leucine‐rich repeat protein 4 (FBXL4) is a mitochondrial protein whose exact
function is not yet known. However, cellular studies have suggested that it plays significant …

Abstract Mutations in FBXL4 (F-Box and Leucine rich repeat protein 4), a nuclear-encoded
mitochondrial protein with an unknown function, cause mitochondrial DNA depletion …

Mitochondrial DNA depletion syndromes (MTDPS) are a group of rare genetic disorders
caused by defects in multiple genes involved in mitochondrial DNA (mtDNA) maintenance …