PZL-A

PZL-A is a activator of mitochondrial DNA (mtDNA) synthesis. PZL-A restores wild-type-like activity to mutant forms of polymerase γ (POLγ) with 160 nM for A467T and 20 nM for G848S. In cellular systems, PZL-A stimulates mtDNA synthesis, promoting oxidative phosphorylation complex biogenesis and improving cellular respiration. PZL-A is promising agent for relieving POLG disease and other severe conditions linked to depletion of mtDNA.