This transgenic strain carries the normal allele of the human SOD1 gene. Gurney ME, Pu H, Chiu AY, Dal Canto MC, Polchow CY, Alexander DD, Caliendo J, Hentati A, Kwon YW, Deng HX, Chen W, Zhai P, Sufit RL, Siddique T. 1994. Please note that use of these mice for commercial purposes or by a for-profit entity may require a license from the originating institution. T his transgenic strain carries the normal allele of the human SOD1 gene. Please note that use of these mice for commercial purposes or by a for-profit entity may require a license from the originating institution.
Transgenic mice develop a motor neuron disease which is severe and progressive.
Onset of the disease on the B6C3F1 genetic background is between 6 and 8 months. Early signs of disease are reduced spontaneous movement and difficulty moving hind limbs when lifted by the tail. This progresses to asymmetric weakness of limbs, poor grooming, rough coat, muscle wasting, and paralysis of the hind limbs. There is a progressive vacuolation seen in both axons and dendrites, which appears to be caused by degenerating mitochondria. It is currently being maintained by crossing hemizygous carriers to B6C3F1 animals.