The assembly pathway of the mammalian mitochondrial ribosome
Mitochondria, which fulfill many essential roles within the cell, still contain their own genome (mtDNA). However, the expression of mtDNA-encoded proteins depends on factors encoded by the nuclear genome. Defects in these factors lead to severe mitochondrial diseases in human. This is also true for the mitochondrial ribosomal proteins, which need to be imported into mitochondria, where they assemble with mtDNA-encoded ribosomal RNAs.
We are interested in how the mammalian mitochondrial ribosome assembles from components of dual genetic origin. Although the mitochondrial ribosome derived from a bacterial ancestor, there are substantial differences in structure and composition. Therefore it is reasonable to speculate that the assembly pathways comparing bacterial and mammalian mitochondrial ribosomes vary as well. Our analyses will also help us to understand the diverse clinical presentations of the growing group of patients with mutations in genes encoding for mitochondrial ribosomal proteins.