The autosomal recessive HIBM type 2 (HIBM2) is caused by mutations in the gene encoding glucosamine (UDP-N-acetyl)-2-epimerase/N-acetylmannosamine kinase (GNE) [5], and is allelic with distal myopathy with rimmed vacuoles (DMRV), also known as Nonaka myopathy (NM) [6-10]. The gene discussed is GNE; the disease is distal myopathy.