This PDF file includes:

• table S1. Echocardiographic data of Fbln4^E57K/E57K and littermate WT mice.
  
• fig. S1. Fbln4^E57K/E57K mice are similar in size to their littermates.
  
• fig. S2. Fbln4^E57K/E57K ascending aortae have increased medial wall thickness.
  
• fig. S3. Fbln4^E57K/E57K ascending aortae do not develop calcification.
  
• fig. S4. Fbln4^E57K/E57K mice have similar heart rates to their heterozygous and WT littermates.
  
• fig. S5. Internal elastic lamina is unaffected in Fbln4^E57K/E57K saphenous arteries.
  
• fig. S6. Homozygous E57K mutation in Fbln4 does not alter arterial elastin or collagen content.
  
• fig. S7. Homozygous E57K mutation in Fbln4 does not alter ECM gene expression in lungs.
  
• fig. S8. FBLN4(E57K) protein level is reduced in P7 ascending aorta, but not in mesenteric arteries.
  
• fig. S9. FBLN4(E57K) protein secretion is impaired compared to WT FBLN4.

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