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. 2018 Apr 9;11(4):dmm031542. doi: 10.1242/dmm.031542

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© 2018. Published by The Company of Biologists Ltd

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution and reproduction in any medium provided that the original work is properly attributed.

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Fig. 1. — Generation and MPL phenotype characterization of FBN1 Het rabbits. (A) Design of sgRNAs targeting the C-terminus of the rabbit FBN1 gene. Blue bases represent exon 65 of FBN1; target sites of the two sgRNA sequences, sgRNA1 and sgRNA2, are highlighted in green; protospacer-adjacent motif (PAM) sequence is highlighted in red; ‘RGRKRR’ with gray background is the furin enzyme recognition site. (B) Photograph showing the bent ears, thin face and eyelid abnormalities of a FBN1 Het rabbit. (C) Survival curves show the high mortality of FBN1 Het rabbits (WT, n=9; KO, n=26). (D) Weight comparison results showing growth retardation in the FBN1 Het rabbits (n=6). (E) Photographs showing the swollen lungs of FBN1 Het rabbits (left, F0-9) compared with those of WT rabbits (right). (F) H&E stain showing pulmonary infection and edema (arrows) in an FBN1 Het rabbit (F0-9). Data are presented as mean±s.e.m. and analyzed using Student's t-tests with GraphPad Prism software 6.0. *P<0.05; **P<0.01; ***P<0.001. Scale bars: 50 μm.