Figure 6

Stability profiles of lamin A/C and emerin in lymphoblastoid cells culture after treatment with lovastatin or cycloheximide. To determine the stability profiles of lamin A/C and emerin, cells were grown in the presence or absence of lovastatin (panel A) or cycloheximide (panel B). In A, lymphoblastoid cells with the mutation LMNA R377H (lanes 4–6) and control (lanes 1–3) were grown in the presence of 10 µM lovastatin for 24 h (lanes 2, 5), for 48 h (lanes 3, 6) or in the absence of lovastatin (lanes 1, 4). In B, lymphoblastoid cells with the mutation LMNA R377H (lane 4) and control (lane 2) were grown in the absence (lanes 1, 3) or presence of 50 µg/ml of cycloheximide (lanes 2, 4). Whole cell extracts were resolved on SDS-PAGE. Proteins were transferred to nitrocellulose and immunoblotted with the mab R27 to detect lamins A/C and with the mAb NCL-emerin to detect emerin on the same stripped nitrocellulose. Significantly, lamin A from AD-EDMD patient is instable (compare panel A lanes 5, 6 with lanes 2, 3 and also panel B lane 4 with lane 2). Molecular mass standards are indicated in kDa.