Figure 7.

The Nup155 R391H mutation that causes human cardiac arrhythmia abrogates binding of its normal nucleoporin partners and rim localization. U2OS 2–6–3 cells were transfected with a LacI-CFP construct of full-length wild type human Nup155 and the R391H mutant version of the same construct. The transfected cells were tested by immunofluorescence for colocalization of Nup53 or Pom121 with the wild type or mutant Nup155. Note that Nup53 and Pom121 were the only tested nucleoporins that had been seen to be recruited to wild type LacI-CFP-Nup155 (see Table 1). Here Nup53 recruitment can be observed with wild type LacI-CFP-Nup155 (A), but not with the R391H mutant Nup155 (B). (C) Quantitation for the level of Nup53 and Pom121 colocalization and rim targeting is shown for the wild type and mutant Nup155 constructs.