Mutations in fukutin-related protein (FKRP) give rise to mild and more severe forms of
muscular dystrophy. FKRP patients have reduced glycosylation of the extracellular protein
dystroglycan, and FKRP itself shows sequence similarity to glycosyltransferases, implicating
FKRP in the processing of dystroglycan. However, FKRP localization is controversial, and no
FKRP complexes are known, so any FKRP-dystroglycan link remains elusive. Here, we
demonstrate a novel FKRP localization in vivo; in mouse, both endogenous and …