PPARGC1A and Kearns-Sayre syndrome: This is especially interesting in light of the facts that experimental animals used for modeling cardiomyopathy in Kearns-Sayre syndrome are the tissue-specific knockouts of Tfam [200, 201], a gene under the regulation PGC-1α, and the expression of which is severely downregulated in PGC-1α-deficient mice [119, 175].