Phenotype Image Detail

Image

Caption

Immunohistochemical analysis of glomerular changes in Magi2^tm1Key/Magi2^tm1Key (delta/delta) mice at 10 days, 3 weeks, 5.5 weeks, and 7 weeks of age. (A) Representative light microscopic changes in glomeruli. Whereas glomeruli of wild-type (+/+) mice show no significant light microscopic changes at any age, mutant mice show a progressive glomerulopathy. At 10 days of age, no significant glomerular change is seen. At 3 weeks of age, glomeruli appear mildly hypocellular compared with wild-type mice and show a mild increase in matrix accumulation. By 5.5 weeks of age, the majority of glomeruli show a hyalinized and collapsed tuft associated with a severe extracapillary cell proliferation, in many cases resembling an epithelial cell crescent connecting the glomerulus and Bowman capsule (arrow). No significant glomerular inflammation can be seen. By 7 weeks, most glomeruli are globally sclerosed with hyalinization of the tuft. (B) Synaptopodin (Synpo) expression in glomeruli. Wild-type mice show preserved synaptopodin staining at all ages. At 10 days of age, no significant reduction in synaptopodin expression is seen in mutant mice. As early as 3 weeks of age, a mild reduction of synaptopodin can be seen, with a complete loss of synaptopodin staining in all glomeruli with extracapillary proliferations at 5.5 weeks of age. Of note, epithelial cells within the proliferations are negative for synaptopodin (arrows). No synaptopodin staining is detected in globally sclerosed glomeruli. (C) PAX2 expression in glomeruli. Wild-type mice show PAX2 staining limited to small, flat PEC nuclei lining Bowman capsule (arrows) at all ages. Mutant glomeruli show similar distribution of PAX2 at 10 days and 3 weeks of age (arrows). All cells composing the extracapillary proliferations (arrowheads) are positive for PAX2 at 5.5 weeks. Only occasional PAX2-positive cells are seen in globally sclerosed glomeruli at 7 weeks of age. (Scale bars: 20 um.)

Copyright

This image is from Balbas MD, Proc Natl Acad Sci U S A 2014;111(41):14876-81. Copyright 2014 National Academy of Sciences, U.S.A. J:213553

Associated Alleles

Symbol	Name
Magi2^tm1Key	membrane associated guanylate kinase, WW and PDZ domain containing 2; targeted mutation 1, Charles L Sawyers

Associated Genotypes

Allelic Composition	Genetic Background
Magi2^tm1Key/Magi2^tm1Key	involves: 129S4/SvJae