Phenotype Image Detail

Image

Caption

Comparison of spinal cord pathology in 130-day-old Tg(SOD1)2Gur/0 (Panels A-E) and Tg(SOD1-G93A)1Gur/0 (Panels F-J) mice and in the human Amyotrophic lateral sclerosis (ALS) spinal cord (Panels K-O). Staining is done with the following: rabbit anti-NFL polyclonal antiserum (NFL, Panels A,F,K), alpha-internexin mAb (INT, Panels B,G,L), rabbit antiperipherin polyclonal antiserum (PPH, Panels C,H,M), ubiquitin mAb (UBI, Panels D,I,N), and GFAP mAb (Panels E,J,O). Note that pathology in Tg(SOD1-G93A)1Gur/0 mice resembles that in human ALS. The alpha-internexin mAb stains the spheroids in human ALS less strongly than in the transgenic mice (arrow in L), and one spheroid is not stained (arrowhead in L). The Tg(SOD1)2Gur/0 mice also develop spheroids similar to those seen in Tg(SOD1-G93A)1Gur/0 mice and human ALS. Neurofilament and alpha-internexin colocalize in the same spheroids (arrowheads in F and G). However, ubiquitin and GFAP antibodies barely stain the anterior horns of the Tg(SOD1)2Gur/0 mice but strongly stain the Tg(SOD1-G93A)1Gur/0 mice and the human ALS. Some of the reactive astrocytes in the Tg(SOD1-G93A)1Gur/0 mice are extremely hypertrophic and contain inclusion-like GFAP immunoreactive material (Panel E, insert). All sections were counterstained with hematoxylin.

Copyright

This image is from Tu PH, Proc Natl Acad Sci U S A 1996 Apr 2;93(7):3155-60. Copyright 1996 National Academy of Sciences, U.S.A. J:76718

Associated Alleles

Symbol	Name
Tg(SOD1)2Gur	transgene insertion 2, Mark E Gurney
Tg(SOD1*G93A)1Gur	transgene insertion 1, Mark E Gurney

Associated Genotypes

Allelic Composition	Genetic Background
Tg(SOD1*G93A)1Gur/0	involves: C57BL/6 * SJL
Tg(SOD1)2Gur/0	involves: C57BL/6 * SJL