Extended Data Fig. 9: Early intervention with SMC-specific AAV9-mediated Tfam delivery mitigates the progression of aortic dilation and stiffening in SMC-Notch1−/− mice.
From: Correcting mitochondrial loss mitigates NOTCH1-related aortopathy in mice
(a) Experimental design of AAV9-Tfam treatment (n=7). (b) Statistical analysis of diameters of mice aortic root, ascending aorta, and aortic arch. (c) Statistical analysis of diameters of mice abdominal aorta. (d) Statistical analysis of pulse wave velocity and circumferential cyclic strain of the mice aortas. For comparison, data from groups of control (n=8) and SMC-Notch1−/− mice without (n=9) and with AAV9-Mfn1/2 treatments (n=7) were included. (e) Representative histologic staining with HE, EVG and Alcian blue in the mice aorta. (f) Quantification of aortic medial thickness and elastin breaks (SMC-Notch1−/−, n=5 and SMC-Notch1−/−+AAV-Tfam, n=7). (g) Western blots of the aortas of mice subjected to boosting TFAM treatments by SMC-specific AAV9-Tfam or not (n=3). (h) Flow cytometry analyses of TOMM20. Five mice thoracic aortas were combined for one test of TOMM20 staining and flow cytometry. The ultrasound and histological data of control and SMC-Notch1−/− mice without and with AAV9-Mfn1/2 treatments in this Extended Data Fig. 9b–f and main text Fig. 3c–g were identical (that is, representing the same data) for comparison. Thoracic aortic diameter is presented in box and whisker plots showing maximal and minimal values and 75th and 25th percentiles. Histograms show mean± standard deviation. Statistical significance was assessed by Multiple t tests-one per row (with Holm-Sidak correction) (b) and two-tailed Studentʼs t-tests (c,d,f,g). * represents P<0.05, ** represents P<0.01, *** represents P<0.001, **** represents P<0.0001. All mice were male and subjected to tamoxifen injection. Uncropped western blots and source data are provided as a Source Data file.
