Phenotype Image Detail

Image

Caption

(A-F) H&E staining of cross-sections of muscle fibers in 3, 9 and 18 month old mice. There were no patological changes in 3 month old Tg(Ckm-GSN*D187N)AJewe/0 mice (A). By 9 months, there was excessive variability in myofiber size were atrophic fibers having a basophilic appearance (B, arrows). At 18 months, atrophic fibers and fibers containing internal nuclei. basophilic inclusions (C, arrows; Inset, arrows), and vacuoles (C, arrowheads) were prominent. WIth the exception of small sarcoplasmice vacuoles that are commin in aged mice (F, arrowheads), no histological abnormalities were found in wild-type (wt) mice [3 months(D), 9 months (E), and 18 months (F)]. The asterisk (*) indicates the normal myofiber nuclei that are found toward the periphery of the cell. (G-L) H&E stained in Tg(Ckm-GSN*D187N)AJewe/Tg(Ckm-GSN*D187N)AJewe mice revealed severe myopathic changes at an earlier age (G, 12 months; H, 18 months) including numerous atrophic fibers, endomysial amyloidogenesis, vacuoles (G, black arrows) and fatty infiltration (G, arrowheads), basophilic sarcoplasmic inclusions (H, arrowhead), and foci of mononuclear cell infiltration (G and H, white arrows). CR positivity was extensive (I), including endomysial and sarcoplasmic deposits (I, arrowhead) (H and I, same cells are shown). Electron microscopy showed distended mitochondria, some with inclusions (J, arrow), vacuoles containing tubulomembranous structures and myeloid bodies (K and L, arrowheads), and sarcoplasmic fibrillar deposits (L, arrow). (Scale bars: A-G, 100 um; H and I, 25 um; J, 2.9 um; K, 1.7 um; L, 1.4 um).

Copyright

This image is from Page LJ, Proc Natl Acad Sci U S A 2009 Jul 7;106(27):11125-30. Copyright 2009 National Academy of Sciences, U.S.A. J:150825

Associated Alleles

Symbol	Name
Tg(Ckm-GSN*D187N)AJewe	transgene insertion A, Jeffery W Kelly