Phenotype Image Detail

Image

Caption

Pulmonary arteriovenous malformations (AVMs) in Acvrl1^tm2.1Spo/Acvrl1^tm2.1Spo Tg(Acvrl1-cre)L1Spo/0 mutants. A, B. Gross morphology of P3 lungs. Abnormally dilated and tortuous vessels (arrows) were visible in the mutant lungs (B). C-F. Pulmonary vascular morphologies by the latex dye injection via right heart. The dye visualized arterial branches in the control lungs (C, E), whereas it crossed to venous vessels in the mutants (D, F). Boxes in C and D indicate the areas magnified in E and F, respectively. Nidus-like tangled vessels were indicated by red arrows (F). H, heart; LPa, left pulmonary artery; RPa, right pulmonary artery, Pv, pulmonary vein. G, H. H&E staining of lung sections demonstrates abnormal vascular branches with severe dilations and signs of fusions between vessels (asterisks). I, J. Staining of lung sections with anti-alpha-SMA antibodies outlines pulmonary vessels. Vascular smooth muscle layers in the mutants (J) are thin, irregular and discontinuous indicated by arrows in the inset (J). Br, bronchus; PA, pulmonary artery; PAVM, pulmonary AVM. K, L. Fluorescence microscopic view of Evans Blue dyes in the control (K) and mutant (L) lungs. Arrows indicate the dye found in the airways. Insets show gross morphology of lungs after Evans Blue dye injection. Scale bars indicate 1 mm (A-D), 300 um (E, F), 100 um (I-L), 50 um (insets in I, J), and 500 um (insets in K, L).

Copyright

This image is from Park SO, J Clin Invest 2009 Nov;119(11):3487-96 and is displayed with the permission of the American Society for Clinical Investigation who owns the Copyright. J:154620

Associated Alleles

Symbol	Name
Acvrl1^tm2.1Spo	activin A receptor, type II-like 1; targeted mutation 2.1, S Paul Oh
Tg(Acvrl1-cre)L1Spo	transgene insertion L1, S Paul Oh

Associated Genotypes

Allelic Composition	Genetic Background
Acvrl1^tm2.1Spo/Acvrl1^tm2.1Spo Tg(Acvrl1-cre)L1Spo/0	involves: 129 * FVB