Originally identified in Alberta Dariusleut Hutterites, DnaJC19 mutations are clinically characterized by raised levels of 3-methylglutaconic acid, a readout of mitochondrial distress, dilated cardiomyopathy, prolongation of the QT interval in the electrocardiogram and cerebellar ataxia (Davey et al., 2006). This evidence concerns the gene DNAJC19 and cerebellar ataxia.