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Chair's Introduction (R. Goldman). <p>Nuclear lamins: building blocks of nuclear structure and function (R. Goldman, et al.).</p> <p>Aspects of nuclear envelope dynamics in mitotic cells (B. Burke, et al.).</p> <p>Components of the nuclear envelope and their role in human disease (H. Worman).</p> <p>Nuclear membrane protein emerin: roles in gene regulation, actin dynamics and human disease (K. Wilson, et al.).</p> <p>Identification of novel integral membrane proteins of the nuclear envelope with potential disease links using subtractive proteomics (E. Schirmer, et al.).</p> <p>Genetics of laminopathies (R. Ben Yaou, et al.).</p> <p>Muscular dystrophies related to the cytoskeleton/nuclear envelope (K. Nowak, et al.).</p> <p>Skeletal and cardiac muscle defects in a murine model of Emery-Dreifuss muscular dystrophy (M. Grattan, et al.).</p> <p>Multiple pathways tether telomeres and silent chromatin at the nuclear periphery: functional implications for Sir-mediated repression (A. Taddei, et al.).</p> <p>A-type lamin-linked lipodystrophies (C. Vigouroux and J. Capeau).</p> <p>Cytoskeletal defects in amyotrophic lateral sclerosis (motor neuron disease) (J. Julien, et al.).</p> <p><i>LMNA</i> mutations in progeroid syndromes (S. Huang, et al.).</p> <p>A genetic approach to study the role of nuclear envelope components in nuclear positioning (D. Starr and M. Han).</p> <p>General Discussion I.</p> <p>A lamin-dependent pathway that regulates nuclear organization, cell cycle progression and germ cell development (A. Margalit, et al.).</p> <p>Mutations in the mouse <i>Lmna</i> gene causing progeria, muscular dystrophy and cardiomyopathy (S. Kozlov, et al.).</p> <p>The nuclear membrane and mechanotransduction: impaired nuclear mechanics and mechanotransduction in lamin A/C-deficient cells (J. Lammerding and R. Lee).</p> <p>Chair's summing up (R. Goldman).</p> <p>Index of contributors.</p> <p>Subject index.</p>

"…an excellent guide to research in the field of nuclear organization, and an encouragement for students interested in learning how great minds tackle complex problems." (<i>Doody's Health Services</i>)

<b>The Novartis Foundation</b> is an international scientific and educational charity which promotes the study and general knowledge of science and in particular encourages international co-operation in scientific research.<br /> Chair: <b>Robert D. Goldman</b>

The nuclear envelope is the boundary between a cell’s nucleus and the surrounding cytoplasm, and consists of inner and outer membranes traversed by the nuclear pores.  Underlying the inner nuclear membrane is a thin proteinaceous layer, the nuclear lamina, which comprises princiapally nuclear lamins - intermediate filament-type proteins.  This envelope regulates several important processes: the traffic of molecules between the nucleus and the cytoplasm, nuclear morphology during the cell cycle, DNA synthesis and chromatin organization. <p>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.</p> <p>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.</p>

GB