Mitochondria are the major source of ATP in the cell. Five multi-subunit complexes in the
inner membrane of the organelle are involved in the oxidative phosphorylation required for …

The accumulation of protein inclusions is linked to many neurodegenerative diseases that
typically develop in older individuals, due to a combination of genetic and environmental …

Regulation of mtDNA expression is critical for maintaining cellular energy homeostasis and
may, in principle, occur at many different levels. The leucine‐rich pentatricopeptide repeat …

Understanding regulation of mitochondrial DNA (mtDNA) expression is of considerable
interest given that mitochondrial dysfunction is important in human pathology and aging …

The pentatricopeptide repeat (PPR) family plays a major role in RNA stability, regulation,
processing, splicing, translation, and editing. Leucine-rich PPR-motif-containing protein …

In human mitochondria, 10 mRNAs species are generated from a long polycistronic
precursor that is transcribed from the heavy chain of mitochondrial DNA, in theory yielding …

In this contribution we summarize most of the findings reported for the molecular and cellular
biology of the physiological inhibitor of the mitochondrial H+-ATP synthase, the engine of …

Mitochondria maintain genome and translation machinery to synthesize a small subset of
subunits of the oxidative phosphorylation system. To build up functional enzymes, these …

In mammals, the leucine-rich pentatricopeptide repeat protein (LRPPRC) and the stem-loop
interacting RNA-binding protein (SLIRP) form a complex in the mitochondrial matrix that is …

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 …