Mutation in STUB1/CHIP have been found in Gordon Holmes syndrome (multisystemic neurodegeneration; Hayer et al., 2017) and more recently in SCA48 (Genis et al., 2018), and a destabilized CHIP (linked to six different variants) is present in SCA16 (Pakdaman et al., 2017; Kanack et al., 2018); also, a missense mutation in the CHIP-ubiquitin ligase domain was reported as the cause of a form of spinocerebellar autosomal recessive 16 (SCAR16; Shi et al., 2013, 2018). This evidence concerns the gene STUB1 and Cerebellar ataxia - hypogonadism.