{"product_id":"nuclear-organization-in-development-and-disease-isbn-9780470093733","title":"Nuclear Organization in Development and Disease","description":"This book draws together contributions from cell and developmental biologists, structural biologists, geneticists and clinical scientists aimed at a better understanding of the cellular and molecular basis of these diseases. Topics include:  \u003cul\u003e \u003cli\u003eHow nuclear structure and location within a nucleus affect gene expression\u003c\/li\u003e \u003cli\u003eChromatin organization and cell differentiation\u003c\/li\u003e \u003cli\u003eThe nature of the interactions between the nuclear envelope and the cytoskeleton\u003c\/li\u003e \u003cli\u003eThe 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\u003c\/li\u003e \u003c\/ul\u003e \u003cp\u003eIt is hoped that a better understanding of the mechanisms leading to disease pathogenesis may ultimately lead to more rational and appropriate treatments.\u003c\/p\u003e  Chair's Introduction (R. Goldman).  \u003cp\u003eNuclear lamins: building blocks of nuclear structure and function (R. Goldman, et al.).\u003c\/p\u003e \u003cp\u003eAspects of nuclear envelope dynamics in mitotic cells (B. Burke, et al.).\u003c\/p\u003e \u003cp\u003eComponents of the nuclear envelope and their role in human disease (H. Worman).\u003c\/p\u003e \u003cp\u003eNuclear membrane protein emerin: roles in gene regulation, actin dynamics and human disease (K. Wilson, et al.).\u003c\/p\u003e \u003cp\u003eIdentification of novel integral membrane proteins of the nuclear envelope with potential disease links using subtractive proteomics (E. Schirmer, et al.).\u003c\/p\u003e \u003cp\u003eGenetics of laminopathies (R. Ben Yaou, et al.).\u003c\/p\u003e \u003cp\u003eMuscular dystrophies related to the cytoskeleton\/nuclear envelope (K. Nowak, et al.).\u003c\/p\u003e \u003cp\u003eSkeletal and cardiac muscle defects in a murine model of Emery-Dreifuss muscular dystrophy (M. Grattan, et al.).\u003c\/p\u003e \u003cp\u003eMultiple pathways tether telomeres and silent chromatin at the nuclear periphery: functional implications for Sir-mediated repression (A. Taddei, et al.).\u003c\/p\u003e \u003cp\u003eA-type lamin-linked lipodystrophies (C. Vigouroux and J. Capeau).\u003c\/p\u003e \u003cp\u003eCytoskeletal defects in amyotrophic lateral sclerosis (motor neuron disease) (J. Julien, et al.).\u003c\/p\u003e \u003cp\u003e\u003ci\u003eLMNA\u003c\/i\u003e mutations in progeroid syndromes (S. Huang, et al.).\u003c\/p\u003e \u003cp\u003eA genetic approach to study the role of nuclear envelope components in nuclear positioning (D. Starr and M. Han).\u003c\/p\u003e \u003cp\u003eGeneral Discussion I.\u003c\/p\u003e \u003cp\u003eA lamin-dependent pathway that regulates nuclear organization, cell cycle progression and germ cell development (A. Margalit, et al.).\u003c\/p\u003e \u003cp\u003eMutations in the mouse \u003ci\u003eLmna\u003c\/i\u003e gene causing progeria, muscular dystrophy and cardiomyopathy (S. Kozlov, et al.).\u003c\/p\u003e \u003cp\u003eThe nuclear membrane and mechanotransduction: impaired nuclear mechanics and mechanotransduction in lamin A\/C-deficient cells (J. Lammerding and R. Lee).\u003c\/p\u003e \u003cp\u003eChair's summing up (R. Goldman).\u003c\/p\u003e \u003cp\u003eIndex of contributors.\u003c\/p\u003e \u003cp\u003eSubject index.\u003c\/p\u003e \"…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.\" (\u003ci\u003eDoody's Health Services\u003c\/i\u003e)  \u003cb\u003eThe Novartis Foundation\u003c\/b\u003e 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.\u003cbr\u003e Chair: \u003cb\u003eRobert D. Goldman\u003c\/b\u003e  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.  \u003cp\u003eNine 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.\u003c\/p\u003e \u003cp\u003eThis 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.\u003c\/p\u003e","brand":"Wiley","offers":[{"title":"Default Title","offer_id":47989701279973,"sku":"NP9780470093733","price":211.95,"currency_code":"USD","in_stock":false}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/1842\/7735\/files\/9780470093733.jpg?v=1761785162","url":"https:\/\/k12savings.com\/es\/products\/nuclear-organization-in-development-and-disease-isbn-9780470093733","provider":"K12savings","version":"1.0","type":"link"}