With special attention to muscle maintenance in terms of regeneration and atrophy, this review is focused on the roles of TRIM32 and its E3 ubiquitin ligase activity in skeletal muscle; specifically, TRIM32-interacting proteins in skeletal muscle, expression levels of TRIM32 under various conditions, such as cellular senescence and terminal differentiation, and patients with muscular dystrophies and atrophies due to LGMD2H. This evidence concerns the gene TRIM32 and autosomal recessive limb-girdle muscular dystrophy type 2H.