Among the disease proteins responsible for Perrault syndrome, HARS2 was prominent because of its strong accumulation in CLPP-null tissue, as previously reported upon quantitative immunoblots [17], and here, the complexomics profile revealed a parallel distribution of CLPX and HARS2 from their monomeric sizes (precursors of 69 and 23 kDa, respectively) up to molecular sizes around 250 kDa. Here, CLPP is linked to Perrault syndrome.