Genetic Engineering of Dystroglycan in Animal Models of Muscular Dystrophy

Table 1

DG mouse models characterized by a muscle and/or central nervous system phenotype.

Mouse model	Muscular dystrophy	CNS involvement	NMJs
Chimaeric mice [34]	Progressive	—	Disorganized and disrupted
MCK-Cre/DG-null [38]	Mild	—	Normal
GFAP-Cre/DG-null [45]	—	Neuronal migration errors, brain malformation	—
MORE-DG-null [38, 39]	Severe	Neuronal migration errors, brain malformations, and ocular defects (WWS phenotype)	—
Nestin-Cre/DG-null [47] and Crx-Cre/DG-null [50]	—	Abnormal retinal physiology	—
DG^T192M/DG^T192M [21]	Mild	Some neurological impairments	Compromised
DG^Y890F/Y890F [56]	Normal	Normal	Normal
DG^Y890F/Y890F/mdx [56]	Ameliorated	—	Ameliorated
DG^WToverexpression [40]	Normal	Normal	25% smaller than normal but only 1% are aberrant
DG^WToverexpression/mdx [40]	Not ameliorated	—	Not ameliorated
DG^S654Aoverexpression [63]	Mild	—	Compromised
[47]	—	Mild effects in the retina	—

—: not analysed.