Table 1.
Model | Breed | Phenotype | Affected gene | Mutations | Advantages | Disadvantages |
---|---|---|---|---|---|---|
Naturally occurring models | ||||||
Cat7–12 | Maine Coon Ragdoll |
HCM | MYPBC3 | c.91G>C [p.Ala31Pro] c.2328C>T [p.Arg820Trp] |
|
|
Dog13–25 | Port. Waterdogs Great Danes Doberman Boxers |
DCM DCM DCM DCM/ARVC |
Uncertain Uncertain Uncertain Uncertain |
Unknown Unknown Unknown Unknown |
|
- Genes and mutations presently unknown.- Gene targeting is difficult. |
Genetically engineered models | ||||||
Mouse26–40 | HCM HCM DCM |
Mybpc3, Myh6, Tnni3, Tnnt2, Tpm1 Mybpc3, Myh6, Tnnt2 Mlp/Csrp3 |
Transgenesis Knock-in/-out Knock-out |
|
|
|
Rat41 | HCM | Tnnt2 | Transgenesis (truncated protein) |
|
|
|
Rabbit42 | HCM | TNNI3 | Transgenesis |
|
||
Zebrafish43 | DCM | Mybpc3 | [p.Val762Asp/p. Arg820Gln] |
|
|
|
Drosophila44,45 | HCM RCM |
mybpc3 tnnt2 |
Wild-type, truncated up101 TnT1 |
|
|
HCM: hypertrophic cardiomyopathy; DCM: dilated cardiomyopathy; ARVC: arrhythmogenic right ventricular cardiomyopathy; RCM: restrictive cardiomyopathy; Gene abbreviations used: Mybpc3: cardiac myosin-binding protein C; Myh6: α-myosin heavy chain; Myh7: β-myosin heavy chain; Tnni3: cardiac troponin I; Tnnt2: cardiac troponin T; ilk: integrin-linked kinase; mlp/Csrp3: cysteine and glycine-rich protein; not3: CG8426 gene product from the transcript CG8426-RB.