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PZL-A is an allosteric activator of mutant DNA polymerase γ (POLγ), an enzyme frequently mutated in POLG-related mitochondrial disease.1 It stabilizes the interface between the subunits POLγA and POLγB in mutant POLγ and restores polymerase activity with half maximal activity concentration (AC50) values of 0.16 and 0.02 µM for POLγA467T and POLγG848S, respectively. PZL-A restores decreases in mitochondrial DNA (mtDNA) levels induced by ethidium bromide (Item No. 39132) and increases ATP production in fibroblasts isolated from patients with POLγA467T/G848S or POLγW748S/R232H mutations.
WARNING This product is not for human or veterinary use.
1. Small molecules restore mutant mitochondrial DNA polymerase activity. Nature 642(8067), 501-507 (2025).