Review Article

Genetic Engineering of Dystroglycan in Animal Models of Muscular Dystrophy

Table 2

Mouse models in which DG was targeted in tissues other than skeletal muscle and brain and additional DG animal models with muscle and central nervous system defects.

Animal modelPhenotype

Kidney specific DG knock-out mouse (podocin-Cre/DG-null, Pax2-Cre/DG-null, Pax3-Cre/DG-null, HoxB7-Cre/DG-null) [64]Normal
Schwann cells specific DG knock-out mouse (P0-Cre/DG-null) [66] Severe neurological dysfunctions
DG knock-out in Caenorhabditis  elegans [68]Defects in gonad and vulval epithelium and in motoneurons
RNAi knock-out of DG in Drosophila melanogaster [73, 74]Muscle degeneration and neuronal defects
Inhibition of DG translation via morpholino antisense in zebrafish [78]Muscle defects
Zebrafish patchytail [79]Dystrophic muscles, ocular and central nervous system defects
Zebrafish dag1hu3072 [81]Muscular dystrophy
Inhibition of DG translation via morpholino antisense in Xenopus laevis [8286]Defects in the somitogenesis, epidermal differentiation, the retinal and renal developing
Overexpression of DG in Xenopus laevis embryos [86]Aberrant neuromuscular junctions