LGMD, for which a revised classification system has recently been developed [26], includes one subtype, LGMDR9 (formally 2I), which is caused by mutations in the Fukutin-Related Protein gene (FKRP) [27], in which individuals are most commonly homozygous for a Scandinavian founder mutation [28]. Here, FKRP is linked to limb-girdle muscular dystrophy.