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Title:
Nuclear organization in development and disease
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Series:
Novartis Foundation Symposium ; 264
Publication Information:
Hoboken, NJ : Wiley, 2005
ISBN:
9780470093733
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30000010129401 QH595 C42 2005 Open Access Book Book
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Summary

Summary

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: 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 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

It is hoped that a better understanding of the mechanisms leading to disease pathogenesis may ultimately lead to more rational and appropriate treatments.


Author Notes

The Novartis Foundation 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.
Chair: Robert D. Goldman


Table of Contents

Chair2s IntroductionR. Goldman
Nuclear lamins: building blocks of nuclear structure and functionR. Goldman, et al.
Aspects of nuclear envelope dynamics in mitotic cellsB. Burke, et al.
Components of the nuclear envelope and their role in human diseaseH. Worman
Nuclear membrane protein emerin: roles in gene regulation, actin dynamics and human diseasK. Wilson, et al.
Identification of novel integral membrane proteins of the nuclear envelope with potential disease links using subtractive proteomicsE. Schirmer, et al.
Genetics of laminopathiesR. Ben Yaou, et al.
Muscular dystrophies related to the cytoskeleton/nuclear envelopeK. Nowak, et al.
Skeletal and cardiac muscle defects in a murine model of Emery-Dreifuss muscular dystrophyM. Grattan, et al.
Multiple pathways tether telomeres and silent chromatin at the nuclear periphery: functional implications for Sir-mediated repressionA. Taddei, et al.
A-type lamin-linked lipodystrophiesC. Vigouroux and J. Capeau
Cytoskeletal defects in amyotrophic lateral sclerosis (motor neuron disease)J. Julien, et al.
LMNA mutations in progeroid syndromesS. Huang, et al.
A genetic approach to study the role of nuclear envelope components in nuclear positioningD. Starr and M. Han
General Discussion I
A lamin-dependent pathway that regulates nuclear organization, cell cycle progression and germ cell developmentA. Margalit, et al.
Mutations in the mouse Lmna gene causing progeria, muscular dystrophy and cardiomyopathyS. Kozlov, et al.
The nuclear membrane and mechanotransduction: impaired nuclear mechanics and mechanotransduction in lamin A/C-deficient cellsJ. Lammerding and R. Lee
Chair2s summing upR. Goldman
Index of contributors
Subject index