HYOU1 and cerebellar ataxia: In accordance with our finding of decreased HYOU1 in white blood cells derived from GAN patients, Zhao and colleagues in 2010 reported that overexpression of HYOU1 prevents ER stress and rescues neurodegeneration in Sil1(-/-) mice as an in vivo model of cerebellar ataxia [26, 27], whereas decreasing expression of HYOU1 exacerbates this phenotype [28].