AMPK Methods and Protocols

Fig. 2Transgenic mice with cardiac-restricted overexpression of mutantPRKAG2recapitulate the severe end
of the human PRKAG2 cardiomyopathy phenotypic spectrum. Upper row, long-axis sections of hearts from
non-transgenic wild-type (a), TGWT(b), and TGN488I(c) mice, demonstrating marked cardiac hypertrophy
resulting from cardiac-restricted overexpression of an Asn488Ile mutatedPRKAG2transgene (i.e., TGN488I)
and milder hypertrophy resulting from overexpression of WTPRKAG2alone (TGWT); scale bar 2 mm. Second
row, hematoxylin and eosin-stained sections from WT (d), TGWT(e), and TGN488I (f) mice illustrating
cardiomyocyte vacuolation. Third row, PAS-stained sections from WT (g), TGWT(h), and TGN488I(i) mice.
Lower row, cardiac section stained with toluidine blue illustrating large glycogen pools in cardiomyocytes from
TGN488Imice but normal appearances (inset) from WT mice, (j) and transmission electron micrograph of an
TGN488Icardiomyocyte demonstrating free cytosolic glycogen and myofibrillar disruption (k); scale bar 3μm
(Reproduced from ref.32 with permission from Wolters Kluwer Health, Inc.)