Phenotype Image Detail

Image

Caption

130-day-old Tg(SOD1-G93A)1Gur/0 mice contain all three neurofilament (NF) subunits in the NF spheroids. The NFM and NFH subunits are phosphorylated. Panels A-D were stained with rabbit anit-NFL polyclonal anti-serum and the rest of the panels were stained for neurofilaments with the specific antibody indicated. Panel A. Extensive perikaryal vacuolation in affected motor neuron. The asterisk indicates the nucleus. Panel B. Dystrophic neurite in anterior horn. Panel C. Lewy body-like spheroid in anterior horn. Panel D. Spheroid with pale center. Panels E-H are stained for the middle-molecular weight NF (NFM) protein. Panel E. RMO3 is a phosphorylation-independent mAb that recognizes the tail domain outside the multiphosphorylation repeat in the NFM. Panel F. RMO189 is a phosphorylation-independent mAb specific for the NFM rod domain. Panel G. RMO255 is a phosphorylation-independent mAb that recognizes the last 20 amino acids of NFM. Panel H. RMO32 is a phosphorylation-dependent mAb that recognizes the highly phosphorylated multiphosphorylation repeat of NFM. Panels I-L are stained for the high-molecular weight NF (NFH) protein. Panel I. RmdO9 recognizes six consecutive nonphosphorylated Lys-Ser-Pro motifs in the multiphosphorylation repeat of NFH. Panel J. TA51 is a mAb that recognizes a lightly phosphorylated NFH epitope. Panel K. RMO217 is a mAb that recognizes a heavily phosphorylated NFH epitope. All sections are 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*G93A)1Gur	transgene insertion 1, Mark E Gurney

Associated Genotypes

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