Mitochondria play a central role in cellular energy provision. The organelles contain their
own genome with a modified genetic code. The mammalian mitochondrial genome is …

The discovery that mutations in mitochondrial DNA (mtDNA) can be pathogenic in humans
has increased interest in understanding mtDNA maintenance. The functional state of mtDNA …

Mitochondria are intimately involved in the generation of and defense against reactive
oxygen species (ROS). Mitochondria are themselves targets of oxidative stress and also …

The recessively inherited developmental disorder, cartilage-hair hypoplasia (CHH) is highly
pleiotropic with manifestations including short stature, defective cellular immunity, and …

Nuclear ribonuclease (RNase) P is a ubiquitous essential ribonucleoprotein complex, one of
only two known RNA-based enzymes found in all three domains of life. The RNA component …

We have developed techniques for the detailed analysis of cis‐acting sequences in the pre‐
rRNA of Saccharomyces cerevisiae and used these to study the processing of internal …

Ribosomes are perhaps the most critical macromolecular machine as they are tasked with
carrying out protein synthesis in cells. They are incredibly complex structures composed of …

The synthesis of ribosomes is one of the major cellular activities, and in eukaryotes, it takes
place primarily, although not exclusively, in a specialized subnuclear compartment termed …

RNase MRP is a site-specific ribonucleoprotein endoribonuclease that cleaves RNA from
the mitochondrial origin of replication in a manner consistent with a role in priming leading …

Proper regulation of ribosome biosynthesis is mandatory for cellular adaptation, growth and
proliferation. Ribosome biogenesis is the most energetically demanding cellular process …