Table 1.

Distribution of transfected lamin A mutants in MEFs (lmna−/−) and the ability of transfected lamin A to relocate endogenous emerin from ER to the nuclear rim

Plasmid/mutationAssociated disease*Transfection efficiency (%)Transfected cells that are `strong transfectants' (%)Transfected cells with large nuclear foci (%)§Correctly identified as transfectants (%)**
pSVK3/wild-type3838072
L85RCMDIA36241.542††
N195KCMDIA242511.942††
E358KAD-EDMD403011.171
M371KAD-EDMD40259.874
R386KAD-EDMD22297.641††
R453WAD-EDMD37381.149††
W520SAD-EDMD4046050††
R527PAD-EDMD34452.245††
pcDNA4/wild-type4958095
R482QFPLD3848090
  • 400 transfected cells from two separate experiments were examined for each plasmid.

  • * CMDIA, dilated cardiomyopathy; AD-EDMD, autosomal dominant Emery-Dreifuss muscular dystrophy; FPLD, familial partial lipodystrophy.

  • Percentage of the total cells examined that were transfected.

  • Percentage of transfected cells that expressed high levels of transfected lamin A.

  • § Percentage of transfected cells that showed large (>0.7 μm) nuclear foci of transfected lamin A.

  • Significantly greater numbers of nuclear foci than wild-type (χ2 test; P<0.01).

  • ** Values shown were obtained from two separate transfection experiments. For each plasmid, 200 strong transfectants were studied as described in Materials and Methods. The values are the percentage of lamin A transfectants that could be correctly identified from the redistribution of emerin alone.

  • †† Significantly less able to relocate emerin to the nuclear rim compared with appropriate wild-type (χ2 test, P<0.001).