|
DM500
|
human DM1 locus with ~500 CTG triplets |
human DMPK promoter/all DM1 related tissues (e.g., skeletal muscle, heart, CNS) |
homozygous mice: myopathy, reduced muscle strength, myotonia (generally very mild phenotype) |
[28,29] |
|
DMSXL
|
human DM1 locus with ~1600 CTG triplets |
human DMPK promoter/all DM1 related tissues (e.g., skeletal muscle, heart, CNS) |
homozygous mice: myopathy, reduced muscle strength, myotonia, reduced body size (more severe phenotype than DM500 mice) |
[30] |
|
HSA
LR
|
human α-actin gene with ~250 CTG triplets |
human α-actin promoter/ skeletal muscle only |
homozygous mice: strong myotonia, myopathy, no muscle weakness |
[27] |
|
mdx
|
spontaneous point mutation in dystrophin gene |
ubiquitous (e.g., skeletal muscle, heart, CNS, retina) |
hemizygous mice: muscle degeneration and atrophy, skeletal muscle fibrosis and necrosis |
[32,33] |
|
WT
|
no transgene (genetic background >90% C57BL/6) |
n.a. |
n.a. |
n.a. |
