Mutations in the chaperone DNAJC19 (encoded by the DNAJC19 gene and homolog of Pam18) have been associated with dilated cardiomyopathy with ataxia (DCMA) (IVS3-1G>C, c.300delA resulting in a p.A100fs*11 frameshift) (see Table 1) [67,68], as well as in combination with progressive cerebellar atrophy (c.280+1_280+5delGTAAG) [69] or with sensorineural hearing loss and bilateral basal ganglia lesions (p.Tyr21*) [70]. Here, DNAJC19 is linked to Ataxia.