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. 2002 Jun 15;22(12):4825–4832. doi: 10.1523/JNEUROSCI.22-12-04825.2002

Fig. 1.

Fig. 1.

Establishment of transgenic mouse lines expressing high levels of mutant SOD1(G93A) or SOD1(G85R) in postnatal neurons, including spinal and upper motoneurons. A–C, Thy1-SOD1(G93A) transgenic lines. A, Expression of transgene in lumbar spinal cord (s.cord) neurons [Thy1-SOD1(G93A) line 13, 1 month; EGFP mRNA; arrowspoint to transgene-positive large ventral horn neurons]. In control experiments, when the EGFP probe was applied to sections from nontransgenic mice, it yielded no detectable signals at up to fivefold longer color development times (data not shown). B, Expression of transgene in motor cortex neurons [Thy1-SOD1(G93A) line 13, 1 month; EGFP mRNA; arrow points to transgene-positive layer 5 neurons]. C, Top three rows, Immunoblots of human (h) and mouse (m) SOD1 in 1 month lumbar spinal cord homogenates (top two rows) and 1 month L3–L5 ventral roots from wild-type (wt), hMg-SOD1(G93A) high-expression line (hMg) (Gurney et al., 1994), and Thy1-SOD1(G93A) transgenic lines 1, 11, 13, and 16. Bottom row, SOD activity in 1 month spinal cord homogenates from wild-type (left) and Thy1-SOD1(G93A) 13 mice (right, brain homogenate). Note that on this nondenaturing gel, human SOD1 migrated faster than endogenous mouse SOD1. D–F, Thy1-SOD1(G85R) transgenic lines. D, E, Expression of transgene in lumbar spinal cord (D) and motor cortex layer 5 (E) neurons (1 month mouse, details as inA and B). F, Immunoblot of human and mouse SOD1 in lumbar spinal cord homogenates (1 month) from Thy1-SOD1(G85R) transgenic lines 5 and 6. G–I, EGFP fluorescence in peripheral nerves from a wild-type (G) and Thy1-SOD1(G93A) line 13 mouse (H, I; 14 months). G, Level of peroneal nerve (as inI). H, Level of the tibial nerve, where three muscular branches arise that run along the external sural (arrow), internal sural, and posterior tibial vessels, respectively. I, Level of common peroneal nerve, where it divides into superficial (arrow) and deep peroneal branches. J, Hindlimb muscle strength as a function of age in wild-type, Thy1-SOD1(G93A) line 13, Thy1-SOD1(G85R) line 6, and high-expressing hMg-SOD1(G93A) mice. The values are averages from three mice each.