The LGMD2H-causing TRIM32 D489N variant (which corresponds to D487N in patients with LGMD2H) expressed in mouse epidermal keratinocyte cells or evaluated via in vitro ubiquitination assays failed to bind to PIASy, to ubiquitinate PIASy or to undergo autoubiquitination [61]. Here, PIAS4 is linked to autosomal recessive limb-girdle muscular dystrophy type 2H.