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. 2020 Aug 7;3(3):237–244. doi: 10.1002/ame2.12129

TABLE 1.

Comparison of ALS‐related behavioral features of four published C9 BAC transgenic mouse models and our KI rats

Model Size of human C9orf72 DNA fragment Human DNA transcript C9orf72 protein levels Motor neuron loss Motor deficits Hindlimb paralysis
A:BAC(G4C2)500 transgenicC57BL/6J 14 BAC DNA including exon1‐5, 800 G4C2 repeats and 141Kb of promoter region. Mix of 300‐500 repeats expressed at similar levels to human. No change of the endogenous C9orf72 protein. Undetected Undetected Undetected
B: BAC(G4C2)100‐1000 transgenic C57BL/6J 15 BAC DNA including 800 G4C2 repeats and complete C9orf72 gene. Mix of 100‐1000 repeats expressed at similar levels to human. C9orf72 protein increased from transgenic mRNA. Undetected Undetected Undetected
C: BAC(G4C2)450 C57BL6/C3H 16 BAC DNA including exon1‐5, 450 G4C2 repeats and 141Kb of promoter region. 450 repeats expressed up to 8‐fold higher than human levels. No change of the endogenous C9orf72 protein. Undetected Undetected Undetected
D: BAC(G4C2)500 transgenic FVB/NJ 17 BAC DNA including 830 G4C2 repeats and complete C9orf72 gene. 500 repeats expressed at similar levels to human. C9orf72 protein increased from transgenic mRNA. Detected Detected Detected
E: (G4C2)80 knockin SD rats DNA including exon1a and exon1b, 80 G4C2 repeats and the flanking regions. (G4C2)80 was derived by endogenous promotor and assumed to be at similar levels to WT rats. C9orf72 protein reduced by 50%. Detected Detected 25% in female rats.