ABSTRACT
The plasma membrane separates the cell interior from the extracellular space by using a lipid bilayer. This lipid bilayer accommodates diverse membrane proteins, including integral membrane proteins such as receptors, ion channels and transporters, as well as certain antigens that are peripherally associated with the membrane. Mutations in cystic fibrosis transmembrane conductance regulator (CFTR) affect the number of channels in the plasma membrane, channel activity and the intracellular trafficking of CFTR. The nuclear envelope shields the nuclear DNA, mediates the pivotal nucleocytoplasmic exchange of material through nuclear pore complexes (NPCs), is involved in regulation of gene expression and confers essential structural stability to the cell nucleus through the underlying nuclear lamina. The NPC basket is indispensable for the nucleocytoplasmic cross-talk. All in all, degradation of the NPC basket and the nuclear lamina disrupts the essential cross-talk between the chromatin and the nuclear envelope as well as between the nucleo- and cytoplasma.
