In contrast to our results with CaMKII-Cre-driven XRCC1 KO, Nestin-Cre-mediated KO of neuronal XRCC1 has been shown to cause profound neuropathology characterized by mild ataxia, episodic spasms and seizures due to the loss of cerebellar interneurons and a 25% smaller body weight compared to controls [32, 44, 45]. The gene discussed is XRCC1; the disease is cerebellar ataxia.