Possible role of autoantibodies in the pathophysiology of GM2 gangliosidoses
J. Clin. Invest. Akira Yamaguchi, et al. 113:200 doi:10.1172/JCI19639 [Go to this article.]

Figure 5
Amelioration of motor defects in Hexb^–/–FcRγ^–/– mice. (a) When suspended by their tails, Hexb^–/–FcRγ^+/+ mice flexed their limbs, whereas Hexb^–/–FcRγ^–/– mice and Hexb^+/+FcRγ^+/+ mice extended their limbs. (b) Clinical scores were determined by the degree of neurological severity and counted as follows: 0 = normal, 1 = slight, 2 = mild, 3 = moderate, 4 = severe. Hexb^+/+FcRγ^+/+, Hexb^–/–FcRγ^+/+, and Hexb^–/–FcRγ^–/– mice were tested from 10 to 15 weeks. n = 5–10. *P < 0.05, Student’s t test (Hexb^–/–FcRγ+/+ mice vs. Hexb^–/–FcRγ^–/– mice). Squares, Hexb^–/–FcRγ^+/+ mice; diamonds, Hexb^+/+FcRγ^+/+ mice; triangles, Hexb^–/–FcRγ^–/– mice. (c) Rotorod testing was performed by counting the mean time to fall from the rotating rod. n = 5–10. *P < 0.05, Student’s t test (Hexb^–/–FcRγ^+/+ mice vs. Hexb^–/–FcRγ^–/– mice). Squares, Hexb^–/–FcRγ^+/+ mice; diamonds, Hexb^+/+FcRγ^+/+mice; triangles, Hexb^–/–FcRγ^–/– mice.