Biogenese der mitochondrialen Proteinsynthesemaschine

Abstract

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.