Biogenese der mitochondrialen Proteinsynthesemaschine


The biogenesis and function of the mitoribosome is of central importance
as mutations in the respective genes of translation factors,
mitoribosomal proteins and ribosome assembly factors lead to
impaired mitochondrial translation, oxidative phosphorylation deficiency
and subsequently to severe human mitochondrial diseases.
Thus, it is of high interest to understand the biogenesis of the mitoribosome
and to define the assembly factors and their functions within
this complex process.