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. Author manuscript; available in PMC: 2019 Aug 18.
Published in final edited form as: Nat Med. 2019 Feb 18;25(3):423–426. doi: 10.1038/s41591-018-0338-6

Figure 1. CRISPR/Cas9 testing in HGPS cellular models.

Figure 1

(a) sgRNA-LCS1 directs Cas9 nuclease against exon 11 of LMNA gene upstream of the HGPS mutation, disrupting lamin A and progerin without altering lamin C. (b) Cropped Western blot of lamin A, progerin and lamin C from wild-type and LmnaG609G/G609G mouse embryonic fibroblasts (MEFs) transduced with sgRNA-control or sgRNA-LCS1 (n=3 independent infections and MEF lines; two-tailed Student’s t-test). (c) Immunofluorescence analysis of progerin-positive nuclei and quantification of nuclear alterations by 4′,6-diamidino-2-phenylindole (DAPI) staining (n=3 independent infections and MEF lines; two-tailed Student’s t-test). Arrowheads indicate nuclear aberrations. (d) Cropped Western blot of lamin A, progerin and lamin C from wild-type and LMNAG608G/+ human fibroblasts transduced with sgRNA-control or sgRNA-LCS1 (n=3 independent infections; two-tailed Student’s t-test). (e) Progerin immunofluorescence and analysis of nuclear aberrations by DAPI staining (n=3 independent infections; two-tailed Student’s t-test). Arrowheads indicate blebbings and invaginations. Bar plots represent mean ± SD and individual values are overlaid. Scale bars, 40 μm. Uncropped blots are available as Source Data.