Figure 1

A-type lamins are reduced in cells with the mutation LMNA R377H. To determine the level of lamin A/C, lamin B and emerin in AD-EDMD patient 99-3, whole proteins of cell extracts from patient lymphoblastoid (lane 4), myoblast (lane 5), fibroblast (lane 6) cultures and mature muscle (lane 8) or control lymphoblastoid (lane1), myoblast (lane 2) fibroblast (lane 3) cultures and mature muscle (lane 7) were separated by SDS-PAGE, transferred to nitrocellulose and immunoblotted with mab R27 to detect lamin A/C, mab X223 to detect lamin B2, mab NCL-emerin to detect emerin and PEG3 to detect fibrillarin. The same nitrocellulose was used for all antibodies. The antibodies were stripped before the incubation with the next one. The level of lamin A is reduced in lymphoblastoids and fibroblasts cells (see arrows in lanes 4 and 6). Molecular mass standards are indicated in kDa.