POLG and myopathy: Mutations in the POLG gene disrupt mtDNA replication and repair, leading to impaired oxidative phosphorylation, reduced adenosine triphosphate (ATP) production, and increased oxidative stress.[1, 2] These deficiencies are particularly harmful to neurons, which have high energy demands, resulting in progressive neuronal degeneration.[3] This mitochondrial failure drives the neurodegenerative symptoms seen in POLG‐related disorders, such as spinocerebellar ataxia, myopathy, and peripheral neuropathy, presenting a significant challenge for treatment.[4, 5]