Since all circadian gene-mutant mouse models show increased sensitivity to γ-radiation, we conclude that the molecular clock functions in tumor suppression in vivo.
Astrocytes from DS fetal brain tissue and DS mouse models show increased concentration of intracellular calcium [55], [56], altered sensitivity to oxidative stress [57], deficits in mitochondrial energy metabolism [22], [58] and abnormal APP transport and secretion [22].
Vitamin D receptor animal knock-out models show increased anxiety, decreased activity, and muscular and motor impairments, resembling phenotypic models of depression.
Similar observations were made in vivo in other models showing increased median survival of mice treated by everolimus first line compared with sunitinib (Rosa et al, 2013).
Interestingly, recent studied in VAP (P56S) transgenic mice models show increased nuclear levels of ATF4 and CHOP (Aliaga et al., 2013).
These data found further confirmation in two other HD mouse models showing increased striatal BimEL in R6/1 mice at the late stages of disease and in the conditional model Tet/HD94.
All models showed increased risks during the end of July and start of August, the period when the temperature had been at an extremely high level for about two weeks.
Furthermore, these mouse models show increased susceptibility to induced ventricular tachycardia (VT) and sudden cardiac death (SCD), supporting the belief that LQT in RTT underlies sudden death (McCauley et al., 2011).
Adipose tissues from murine obesity models show increased mRNA levels of pro-inflammatory cytokines, which are restored to normal levels after treatment with TUDCA, a chemical chaperone that inhibits ER stress [ 106].
Most of the genes that are differentially expressed between the models show increased expression in YAC128 striata and it is possible that this is due to the increased expression of HTT itself over the endogenous HTT expression.
