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. |