Table 1 Overview of Hippo-related mouse models Mouse line Cardiac phenotype Yap activity References Mst1 Tg (αMHC) Dilated cardiomyopathy, increased apoptosis and no compensatory hypertrophy.
To mimic the clinical features of plectin-associated skin blistering diseases in an animal model, several transgenic mouse lines have been generated.
The standard model mouse fibroblast cell line together with the MTT and NR tests were utilized for evaluating the toxicity of the prepared compounds.
Knock-in mutations in mice modeling HD, Sca1 and FFI primarily affect the striatum, cerebellum and thalamus, respectively (Jackson et al., 2009; Lin et al., 2001; Watase et al., 2002), just as in humans, suggesting that the mouse lines model the corresponding diseases relatively well.
Six genetically engineered mouse lines modelling neurodegenerative diseases and neuropsychiatric disorders were considered.
Using a model mouse lung cancer cell line, TC-1, it was shown that when transfected into cells in culture, the pSIN-β generated dsRNA, and the resultant dsRNA seemed to be pro-apoptotic.
In the future, the pneumonectomy mouse model combined with genetically modified mouse lines, lineage tracing approaches, and single-cell transcriptomic analyses will be powerful tools to shed new lights on the regenerative aspects associated with de novo alveologenesis.
As outlined above, this model consists of three independent mouse lines selectively bred for either high (HR), intermediate (IR) or low (LR) reactivity of the HPA axis (for a detailed description of the model see [30]).
We examined one of the first transgenic mouse lines to model mutant TDP-43-mediated neurodegeneration (Wegorzewska et al., 2009), in which the mutant Ala315Thr TARDBP gene is driven by the mouse prion promoter (Prp-TDP43A315T).
Furthermore, the identification of genetically modified mouse lines that model aspects of human developmental abnormalities offers the possibility of investigating the complex interactions of environmental factors that can affect disease prevalence and severity.
