There are multiple mouse models that recapitulate HHT AVM and telangiectasia created through a global or endothelial cell (ECs)-specific knockout of Eng, Alk1, or Smad4 during the embryonic stage, neonatal period, or adulthood [1,9,10,11,12]. The gene discussed is ACVRL1; the disease is hereditary hemorrhagic telangiectasia.