Nuclear Organization in Development and Disease
(Sprache: Englisch)
With contributions from leading cell and developmental biologists, structural biologists, geneticists, and clinical scientists, this book seeks to foster a better understanding of the mechanisms leading to disease pathogenesis.
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Produktinformationen zu „Nuclear Organization in Development and Disease “
With contributions from leading cell and developmental biologists, structural biologists, geneticists, and clinical scientists, this book seeks to foster a better understanding of the mechanisms leading to disease pathogenesis.
Klappentext zu „Nuclear Organization in Development and Disease “
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.
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.
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.
Inhaltsverzeichnis zu „Nuclear Organization in Development and Disease “
Chair's Introduction (Goldman).Nuclear lamins: building blocks of nuclear structure and function (Goldman et al).
Aspects of nuclear envelope dynamics in mitotic cells (Burke et al).
Components of the nuclear envelope and their role in human disease (Worman).
Nuclear membrane protein emerin: roles in gene regulation, actin dynamics and human disease (Wilson et al).
Identification of novel integral membrane proteins of the nuclear envelope with potential disease links using subtractive proteomics (Schirmer et al).
Genetics of laminopathies (Ben Yaou et al).
Muscular dystrophies related to the cytoskeleton/nuclear envelope (Nowak et al).
Skeletal and cardiac muscle defects in a murine model of Emery-Dreifuss muscular dystrophy (Grattan et al).
Multiple pathways tether telomeres and silent chromatin at the nuclear periphery: functional implications for Sir-mediated repression (Taddei et al).
A-type lamin-linked lipodystrophies (Vigouroux and Capeau).
Cytoskeletal defects in amyotrophic lateral sclerosis (motor neuron disease) (Julien et al).
LMNA mutations in progeroid syndromes (Huang et al).
A genetic approach to study the role of nuclear envelope components in nuclear positioning (Starr and Han).
A lamin-dependent pathway that regulates nuclear organization, cell cycle progression and germ cell development (Margalit et al).
Mutations in the mouse Lmna gene causing progeria, muscular dystrophy and cardiomyopathy (Kozlov et al).
The nuclear membrane and mechanotransduction: impaired nuclear mechanics and mechanotransduction in lamin A/C-deficient cells (Lammerding and Lee).
Chair's summing up (Goldman).
Index of contributors.
Subject index.
Autoren-Porträt
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
Bibliographische Angaben
- 2005, 1. Auflage, 256 Seiten, Maße: 24 cm, Gebunden, Englisch
- Verlag: Wiley & Sons
- ISBN-10: 0470093730
- ISBN-13: 9780470093733
Sprache:
Englisch
Pressezitat
" 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 )
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