... transgene, we show early cerebellar deficits in mitochondrial biogenesis and respiratory chain complexes in this FRDA model. At asymptomatic stages, the levels of PGC-1α (PPARGC1A), the mitochondrial biogenesis master regulator, are significantly decreased in cerebellar homogenates of KIKO mice compared...

... margin for compensation by increasing the expression or stabilization of the protein. However, globally increased mitochondrial biogenesis mediated by, for example, PGC-1α (spargel) would be a plausible compensation strategy. In previous studies, this pathway has been implicated in the partial rescue...