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. 2015 Jul 20;112(31):9698–9703. doi: 10.1073/pnas.1508399112

Fig. 4.

Fig. 4.

BPSM1 mutant mice develop aortic root aneurism and aorto-mitral valve inflammation. (A) Kaplan–Meier curve showing the early death of BPSM1 mutant mice on the BALB/c background (black) compared with WT BALB/c mice (blue) or BPSM1 mutant mice on the C57BL/6 background (red). (B and C) Transverse sections through the heart show inflammation of the valves and the dramatic enlargement of the aortic root (dotted circle) in an affected (+/m) BPSM1 mutant mouse on the BALB/c background compared with a WT control (+/+). (D and E) Longitudinal sections through the heart of 141-d-old unaffected (+/+) and affected (+/m) BPSM1 mice of the C57BL/6 background showing inflammation of the valves and limited enlargement of the aortic root. (F and G) The spectacular valve inflammation present in a 46-d-old homozygous (m/m) affected BPSM1 mouse is completely prevented by the loss of TNFR1, even in a 156-d-old homozygote mutant. (H) Transaortic blood velocity measured by echocardiography shows regurgitation in BPSM1+/m mice.