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MORE ABOUT THIS BOOK
Main description:
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.
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.
Back cover:
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.
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.
Contents:
Chair′s Introduction (R. Goldman).
Nuclear lamins: building blocks of nuclear structure and function (R. Goldman, et al.).
Aspects of nuclear envelope dynamics in mitotic cells (B. Burke, et al.).
Components of the nuclear envelope and their role in human disease (H. Worman).
Nuclear membrane protein emerin: roles in gene regulation, actin dynamics and human disease (K. Wilson, et al.).
Identification of novel integral membrane proteins of the nuclear envelope with potential disease links using subtractive proteomics (E. Schirmer, et al.).
Genetics of laminopathies (R. Ben Yaou, et al.).
Muscular dystrophies related to the cytoskeleton/nuclear envelope (K. Nowak, et al.).
Skeletal and cardiac muscle defects in a murine model of Emery–Dreifuss muscular dystrophy (M. Grattan, et al.).
Multiple pathways tether telomeres and silent chromatin at the nuclear periphery: functional implications for Sir–mediated repression (A. Taddei, et al.).
A–type lamin–linked lipodystrophies (C. Vigouroux and J. Capeau).
Cytoskeletal defects in amyotrophic lateral sclerosis (motor neuron disease) (J. Julien, et al.).
LMNA mutations in progeroid syndromes (S. Huang, et al.).
A genetic approach to study the role of nuclear envelope components in nuclear positioning (D. Starr and M. Han).
General Discussion I.
A lamin–dependent pathway that regulates nuclear organization, cell cycle progression and germ cell development (A. Margalit, et al.).
Mutations in the mouse Lmna gene causing progeria, muscular dystrophy and cardiomyopathy (S. Kozlov, et al.).
The nuclear membrane and mechanotransduction: impaired nuclear mechanics and mechanotransduction in lamin A/C–deficient cells (J. Lammerding and R. Lee).
Chair′s summing up (R. Goldman).
Index of contributors.
Subject index.
PRODUCT DETAILS
Publisher: John Wiley & Sons Ltd (Wiley–Blackwell)
Publication date: October, 2008
Pages: 300
Dimensions: 165.00 x 237.00 x 19.00
Weight: 612g
Availability: Not available (reason unspecified)
Subcategories: Diseases and Disorders, General Practice
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CUSTOMER REVIEWS
" 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." ( Doody′s Health Services)