Animal Model

Similarities were found between Drosophila-lethal Abruptex and human CADASIL because they are precisely analogous at the molecular level and both are genetically dominant. This model was proposed to provide more general insights into the VSMC biology because its mutations also lead to an odd number of NECD cysteine.[18] Recently, transgenic mice in which the SM22a promoter drove the expression of the full-length human Notch3 with the Arg90Cys mutation (one CADASIL archetypal mutation) were generated.[19] These A90C-Tg mice showed GOM deposits, NECD accumulation, and evidence of VSMC degeneration strikingly similar to those observed in CADASIL patients. Vessel changes were systemic but more prominent in the tail arteries. Time course analysis of vessel changes revealed that VSMC damages were present before NECD and GOM accumulation occurred (Fig. 2B and C), and that the first ultrastructural findings were the disruption of normal VSMC anchorages to adjacent extracellular matrix and cells (Fig. 2B). Unfortunately, these A90C-Tg mice showed no evidence of brain damage; nevertheless, they modeled the early stage of the human disease before onset of clinical symptoms. One knows the difficulty in predicting the accuracy with which animals will model a corresponding human disease. The mouse with its short life expectancy and its brain anatomy is actually far from humans. SVD model requires animals with brain macro-scopically similar to that of humans. In the future, these facts must be taken into account in studying diseases similar to those of humans.

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