Recently, a number of genes have been identified that are involved in the O-linked glycosylation of α-dystroglycan (αDG)1, an important component of the dystrophin associated complex that anchors muscle fibres to the extracellular scaffold [1], these genes include fukutin, fukutin related protein, LARGE, POMGnT1, POMT1 and POMT2. Mutations in these genes have been shown to result in the aberrant glycosylation of αDG resulting in a broad spectrum of congenital muscular dystrophies [1]. Here, DAG1 is linked to muscular dystrophy.