Indeed, a similar response, identified first in skeletal muscle of the “deletor” mouse model of MM (Tyynismaa et al, 2010), has been documented with disruptions at each step of mtDNA maintenance and expression, including mtDNA replication (Twinkle KO), mtDNA maintenance (Tfam KO), mtDNA transcription (Polrmt KO), mtRNA stability and processing (Lrpprc KO), and mtRNA translation (Mterf4 KO and Dar2 KO) (Dogan et al, 2014; Kühl et al, 2017) in heart muscle. This evidence concerns the gene LRPPRC and Miyoshi myopathy.