• life span improved relative to mice only carrying Tg(SOD1*G93A)^dl1Gur (J:111782)

• improved relative to mice only carrying Tg(SOD1*G93A)^dl1Gur (J:111782)

• used as a measure of paralysis (J:111782)

• reached end stage paralysis later than mice only carrying Tg(SOD1*G93A)^dl1Gur (J:111782)

• improved relative to mice only carrying Tg(SOD1*G93A)^dl1Gur (J:111782)

• 50% more anterior horn motor neurons in the anterior horn of the spinal cord than in mice only carrying Tg(SOD1*G93A)^dl1Gur (J:111782)

• more myelinated axons in the 5th lumbar anterior roots than in mice only carrying Tg(SOD1*G93A)^dl1Gur (J:111782)

• more innervated end plates than in mice only carrying Tg(SOD1*G93A)^dl1Gur (J:111782)

• in the fibularis and peroneus longus (J:111782)

• average death at 244 days compared to 266 days in mice carrying the transgene alone

• at 7.5 months of age

• accelerated compared to transgene alone

• neuromuscular junctions are disorganized and show imperfect colocalization of nerve and postsynaptic sites

• markedly reduced survival, by about 20%

• severe declines in motor function starting around 5-6 months of age

• reduced

• becomes severe after mice begin to display behavioral symptoms

• decreased numbers of microglia compared with Tg(SOD1*G93A)^dl1Gur mice in facial and hypoglossal nuclei at 8 months of age

• decreased numbers of reactive astrocytes compared with Tg(SOD1*G93A)^dl1Gur mice in facial and hypoglossal nuclei at 8 months of age

• less atrophic facial and hypoglossal motoneurons at 8 months of age

• the mean numbers of facial and hypoglossal motoneurons are larger than that of the Tg(SOD1*G93A)^dl1Gur mice, and are almost the same as that of wild type mice

• decreased numbers of microglia compared with Tg(SOD1*G93A)^dl1Gur mice in facial and hypoglossal nuclei at 8 months of age

• decreased numbers of microglia compared with Tg(SOD1*G93A)^dl1Gur mice in facial and hypoglossal nuclei at 8 months of age

• inability to perform hanging wire test

• first sign of disease is forelimb weakness

• the T3 transgene has marginal effects on disease onset and survival in the G1del mutants; onset is 168 to 197 days with end-stage reached at 210-248 days

• animals show forelimb onset of disease

• increased numbers of microglia in facial and hypoglossal nuclei at 8 months of age

• increased numbers of reactive astrocytes in facial and hypoglossal nuclei at 8 months of age

• lower numbers of facial and hypoglossal motoneurons at 8 months of age

• atrophic facial and hypoglossal motoneurons at 8 months of age

• increased numbers of microglia in facial and hypoglossal nuclei at 8 months of age

• increased numbers of microglia in facial and hypoglossal nuclei at 8 months of age

• most symptomatic animals die at <300 days of age

• inability to perform hanging wire test

• first sign of disease is forelimb weakness

• the T3 transgene has marginal effects on disease onset and survival in the G1del mutants; onset is 168 to 197 days with end-stage reached at 210-248 days

• animals show forelimb onset of disease

• vacuolated mitochondria are sometimes seen in spinal cord of end-stage animals

• mean 266 day

• mice develop neurofilament-rich Lewy-body like inclusions in the perikarya and proximal axons of the spinal cord motorneurons at day 180 that increase with age but are not as prominent as in Tg(SOD1*G93A)1Gur mice

• at day 200, both fast and slow axonal transport are impaired in the ventral root compared to in wild-type axons

• develops at day 200

• at day 250 (J:106420)

• develops at day 200