Nestin-Cre-mediated deletion of XRCC1 in the brain during early embryonic development has been reported to cause profound neuropathology that is characterized by mild ataxia accompanied by 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 NES; the disease is Ataxia.