The dual role of MTG3 during human mitoribosome biogenesis

by Marleen Heinrichs
Doctoral thesis
Date of Examination:2024-10-28
Date of issue:2024-12-05
Advisor:Dr. Ricarda Richter-Dennerlein
Referee:Dr. Ricarda Richter-Dennerlein
Referee:Prof. Dr. Kai Tittmann
Persistent Address: http://dx.doi.org/10.53846/goediss-10918

 

 

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Abstract

English

Mitochondria are the main energy provider of the cell, and because of the endosymbiotic event they arise from, still contain a small independent genome. Only with the vast ATP production through the mitochondrial OXPHOS system, the eukaryotic cell is able to satisfy its enormous energy demand, which is reflected by severe mitochondrial diseases caused by a defective OXPHOS system. Mitochondriopathies are not only caused by dysfunctional OXPHOS subunits, but can also be evoked by an improperly working mitochondrial gene expression machinery, as essential OXPHOS subunits are encoded by the mitochondrial genome. Thus, it is of high importance for the cell to guarantee mitochondrial homeostasis, as well as the efficient operation of the gene expression system, including the mitochondrial ribosome (mitoribosome). Mitoribosome biogenesis is a complex process, as mitoribosomes consist of components from dual-genetic origin. All mitoribosomal proteins (MRPs) are encoded in the nucleus, synthesized in the cytosol and
Keywords: Mitochondria; Ribosome biogenesis; GTPase; Assembly factor; Translation initiation; mtSSU maturation