Hemizygous rats express SOD1G93A in the spinal cord ~8-fold above endogenous SOD1. SOD1G93A is also
expressed across many brain regions as well as peripheral tissues. By end stage, mutant SOD1 levels
accumulate ~16 fold over endogenous, representing a further 2-fold increase in SOD1G93A compared with levels in
young, presymptomatic rats (6 weeks old). SOD1 rats have an average onset of motor neuron disease of 115 days,
typically appearing as hind limb abnormal gait and progressing very quickly (1-2 days) to overt hind limb paralysis,
typically affecting one limb first. Hemizygous rats typically reach end-stage disease an average of 11 days after
onset of symptoms. The rapid decline of the SOD1G93A rats coincides with substantial loss of
spinal cord motor neurons as well as marked increases in gliosis and degeneration of muscle integrity and
: Phenotypic changes have been observed in the SOD1 colony which affect disease onset.
Please see the SOD1 communication
for further details.
Genetic Background: NTac:SD Background
Created by John Kulik at Wyeth. Transgenic Model of SOD1 mediated Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig's Disease). Came to Taconic in April 2002 for David Howland at Wyeth for distribution as an Emerging Model. Joint collaboration between Wyeth and The ALS Association.