Finally, ultrastructural evidence of autophagy and uncleared inclusions are present in a mouse model of hereditary inclusion myopathy (h-IBM), a distal myopathy caused by mutations in the UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase (GNE) gene, which encodes for a bifunctional enzyme involved in sialic acid biosynthesis (Malicdan et al. 2007). Here, GNE is linked to distal myopathy.