In addition to the zebrafish utilized by the Udd's group (Sarparanta et al., 2012) to demonstrate that DNAJB6 was the gene responsible for LGMD1D, very recently the Drosophila melanogaster has been used to establish a mechanistic link between the human genes DNAJB6 and hnRNPA2B1 (causing multi system proteinopathy and autosomal dominant familial amyotrophic lateral sclerosis) (Li et al., 2016). Here, DNAJB6 is linked to autosomal dominant limb-girdle muscular dystrophy type 1D (DNAJB6).