Nine human diseases have been linked to mutations in genes encoding components of the nuclear envelope, mostly in the lamin A gene (LMNA).  These diseases include cardiac and skeletal myopathies (Emery-Dreifuss muscular dystrophy, dilated cardiomyopathy and limb-girdle muscular dystrophy 1B); Dunnigan's partial lipodystrophy and mandibuloacral dysplasia; a peripheral neuropathy; Charcot-Marie-tooth disorder type 2, that affects the myelin sheath; and most recently, the premature ageing condition, Hutchinson-Gilford progeria.  Another disease, Pelger-Huet anomaly, that affects nuclear morphology and skeletal development, is linked to an envelope-associated protein, the lamin B receptor.  Furthermore, a recent study suggested that other diseases may be linked to many newly identified nuclear envelope-associated proteins.

This important book draws together contributions from scientists who are studying these diseases from different perspectives: cell and developmental biologists, structural biologists, geneticists and clinical scientists.  Topics include how nuclear structure and location within a nucleus affect gene expression, chromatin organization and cell differentiation; the nature of the interactions between the nuclear envelope and the cytoskeleton; and the extent to which the cytoskeleton mediates communication between the cell membrane and nucleus in regulating gene expression, and whether disruption of such communication might underlie the disease processes.