To explore the role of SEL1L-HRD1 ERAD in cerebellar ataxia, we generated Sel1LPcp2Cre mice with Purkinje cell–specific deletion of Sel1L by crossing Sel1Lfl/fl mice (7) with a transgenic mouse expressing Cre recombinase driven by the promoter of Purkinje cell protein 2 (Pcp2) (47). This evidence concerns the gene SYVN1 and aceruloplasminemia.