Fig 1: Schematic overview of potential mechanisms in the presence (A) and absence (B) of ClpP. ClpP inactivating variants act via ClpX accumulation on mitochondrial nucleoid/RNA granule components, triggering excess POLDIP2, LRPPRC, GFM1, GRSF1, and mtDNA abundance, as well as an enlarged nucleoid area, as a clear result of this study. Less consistent across cell types and potentially without ClpX interactions, multimerizing enzymes in the matrix also show increased abundance. In parallel, elevated levels of varying molecular chaperones suggest a mitochondrial unfolded protein response and aggregation tendencies. OAT multimer and nucleoid shapes were adapted from Refs. [63,64].