Thus, CerS1ΔskMuscle mice indeed recapitulate the skeletal muscle‐specific partial loss of Cer C18:0 of aged mice and allowed us to investigate the structural and functional consequences of CerS1 deficiency independently of the ataxia phenotype caused by full body deletion of CerS1. In CerS1ΔBrain mice, C18:0 Cer remained unchanged in skeletal muscle, while in brain homogenates C18:0 Cer was markedly reduced (Figure 3d, right). Here, CERS1 is linked to cerebellar ataxia.