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Caption (A) EM analysis of amyloid extracted from 18 month hemizygous Tg(Ckm-GSN*D187N)AJewe/0 muscle. Fibers, labeled with anti-FAF anitbodies, were observed in hemizygous mice. (A, arrowheads indicate the 10-nm gold particles) but not in wild-type siblings. The material obtained from hemizygous muscle comprised primarily the 8-kDa fragment of human D187N plasma gelsolin (see Inset). (B) Schematic of a cross-section of a muscle fiber having the same orientation as C-J. (C-J) Cross-sections of muscle fibers were analyzed in hemizygous mice at 3 months (C and G), 9 months (D and H) and 18 months (E and I), and in 18 month old wild-type controls (F and J). In 3 month old mice, CR deposits were localized exclusively around endomysial capillaries (C, arrows). By 9 months, CR reactivity was present around endomysial capillaries but also extended into the endomysium (D, arrows). At 18 months, CR reactivity surrounded all myofibers with several fibers also showing internal sarcoplasmic deposits (E, arrows). No CR positivity (F) was seen in 18 month old wild-type mice. Electron micrographs confirmed fibrillar deposits in a pericapillary localization in 3 month old hemizygous mice (G, arrow). At 9 months, more extensive fibrillar deposits surrounded capillaries (H, arrows) and also extended into enofmysium. Thick pericapillary and endomysial fibrillar deposits (I, black arrow) were found in 18 month old hemizygous muscle. The large fibrillar deposits were between the sarcolemma (I, white arrows) of adjacent cells and the connective tissue of the endomysium (I, white arrow-head) and were absent in wild-type siblings [J, sarcolemma (arrows); endomysium (arrowhead)]. Capillaries are indicated with an asterisk (*). The high magnification Inset (I) shows the fibrillar nature of the deposits. (Scale bars: A, 100 nm; C-F, 100um; G, 3.3 um; H, 2.2 um; I 2.0 um; Inset, 0.3 um; J, 2.0 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

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last database update
07/23/2014
MGI 5.19
The Jackson Laboratory