In addition to its function in the proteasome system, HSJ1 has been shown to be involved in the recruitment of the autophagy marker protein LC3 to damaged mitochondria aiding in mitochondrial autophagy (mitophagy) (Rose et al., 2011) Recessive mutations in HSJ1 affecting both isoforms can cause CMT2T neuropathy or purely motor AR-dHMN (Gess et al., 2014). This evidence concerns the gene DNAJB2 and neuropathy.