Table 1.
Characteristics of DM1 mouse models used in this study.
| Mouse model | Transgene | Transgene copy number | Promoter | Expression | (CTG)n | Genetic background | References |
|---|---|---|---|---|---|---|---|
| DM500 (DM300-328 line) | Human DM1 locus (43 kb transgene) | 1 | Human DMPK (∼11.5 kb region upstream of main TSS) | All DM1-related tissues (e.g. skeletal muscle, heart and CNS) | 500–600 | >90% C57BL/6 | (23) |
| DMSXL (DM300-328 line) | Human DM1 locus (43 kb transgene) | 1 | Human DMPK (∼11.5 kb region upstream of main TSS) | All DM1-related tissues (e.g. skeletal muscle, heart and CNS) | ∼1300 | >90% C57BL/6 | (24) |
| Tg26 | Human DMPK gene (14 kb transgene) | ∼25 | Human DMPK (∼1.9 kb region upstream of main TSS) | All DM1-related tissues (e.g. skeletal muscle, heart and CNS) | 11 | FVB/n | (25) |
| HSALR (LR20b line) | Human α-actin gene; CTG repeat inserted in 3′ UTR (7.1 kb transgene) | 2 | Human α-actin (∼2.1 kb region upstream of TSS) | Skeletal muscle only | 220–250 | FVB/n | (27) |
| WT | No transgene | n.a. | n.a. | n.a. | n.a. | >90% C57BL/6 | n.a. |
TSS, transcription start site; n.a., not applicable.