Cytochrome c oxidase (COX) deficiency is characterized by a high degree of genetic and
phenotypic heterogeneity, partly reflecting the extreme structural complexity, multiple post …

Mitochondria control life and death in eukaryotic cells. Harboring a unique circular genome,
a by‐product of an ancient endosymbiotic event, mitochondria maintains a specialized and …

Mitochondria, often referred to as the powerhouses of cells, are vital organelles that are
present in almost all eukaryotic organisms, including humans. They are the key energy …

Diagnosing primary mitochondrial diseases is challenging in clinical practice. Although,
defective oxidative phosphorylation (OXPHOS) is the common final pathway, it is unknown …

Mammalian mitochondrial ribosomes (mitoribosomes) synthesize a small subset of proteins,
which are essential components of the oxidative phosphorylation machinery. Therefore, their …

The human mitochondrial genome (mtDNA) regulates its transcription products in
specialised and distinct ways as compared to nuclear transcription. Thanks to its mtDNA …

Transcription of the human mitochondrial genome and correct processing of the two long
polycistronic transcripts are crucial for oxidative phosphorylation. According to the tRNA …

Although the large majority of mitochondrial proteins are nuclear encoded, for their correct
functioning mitochondria require the expression of 13 proteins, two rRNA, and 22 tRNA …

The FASTK family proteins have recently emerged as key post-transcriptional regulators of
mitochondrial gene expression. FASTK, the founding member and its homologs FASTKD1 …

Mitochondria contain the genetic information and expression machinery to produce
essential respiratory chain proteins. Within the mitochondrial matrix, newly synthesized …