Abstract: Numerous neurological disorders negatively impact the nervous system, either through loss of neurons or by disrupting the normal functioning of neural networks. These impairments manifest as cognitive defects, memory loss, behavioral abnormalities, and motor dysfunctions. Decades of research have significantly advanced our understanding of the pathophysiology underlying neurodegenerative diseases, including Alzheimer’s disease (AD), Parkinson’s disease, amyotrophic lateral sclerosis, and others. Loss of dopaminergic neurons in the substantia nigra pars compacta with motor function defect is clinically associated with Parkinson’s disease, whereas the accumulation of amyloid-β plaques and tau neurofibrillary tangles is a main pathological hallmark of AD. Beyond these disease-specific mechanisms, key risk factors, such as aging, genomic stress, and mitochondrial dysfunction contribute broadly to the onset and progression of various neurological disorders (Tiwari and Wilson, 2019; Wilson et al., 2023). However, it is still unclear how these events promote the onset of pathological phenotypes and eventually lead to neuronal cell death. The DNA base excision repair (BER) pathway plays a vital role in the maintenance of genome stability and safeguarding human health by repairing endogenous and exogenous oxidative DNA damage. The BER pathway begins with a glycosylase enzyme that recognizes and removes the damaged base, creating an apurinic/ apyrimidinic site. In the next step, an apurinic/ apyrimidinic endonuclease cleaves the DNA backbone at the apurinic/apyrimidinic site, generating a single-strand break. This is followed by gap filling performed by DNA polymerase β, which inserts the correct nucleotide. Finally, DNA ligase seals the nick, restoring the integrity of the DNA strand.