Nuclear Organization in Development and DiseaseDerek J. Chadwick, Jamie A. Goode John Wiley & Sons, 08.02.2006 - 300 Seiten 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:
It is hoped that a better understanding of the mechanisms leading to disease pathogenesis may ultimately lead to more rational and appropriate treatments. |
Inhalt
| 3 | |
Aspects of nuclear envelope dynamics in mitotic cells | 22 |
Components of the nuclear envelope and their role in human disease | 35 |
Nuclear membrane protein emerin roles in gene regulation actin dynamics and human disease | 51 |
Identification of novel integral membrane proteins of the nuclear envelope with potential disease links using subtractive proteomics | 63 |
Genetics of laminopathies | 81 |
Muscular dystrophies related to the cytoskeletonnuclear envelope | 98 |
Skeletal and cardiac muscle defects in a murine model of EmeryDreifuss muscular dystrophy | 118 |
LMNA mutations in progeroid syndromes | 197 |
A genetic approach to study the role of nuclear envelope components in nuclear positioning | 208 |
General discussion I | 227 |
A lamindependent pathway that regulates nuclear organization cellcycle progression and germcell development | 231 |
Mutations in the mouse Lmna gene causing progeria muscular dystrophy and cardiomyopathy | 246 |
The nuclear membrane and mechanotransduction impaired nuclear mechanics and mechanotransduction in lamin ACdeficient cells | 264 |
Chairs summingup | 279 |
| 281 | |
Multiple pathways tether telomeres and silent chromatin at the nuclear periphery functional implications for Sirmediated repression | 140 |
Atype laminlinked lipodystrophies | 166 |
Cytoskeletal defects in amyotrophic lateral sclerosis motor neuron disease | 183 |
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Häufige Begriffe und Wortgruppen
A-type A-type lamins a¡ect abnormal actin Alsin anchoring antibody assembly associated autosomal binding Bonne broblasts Burke C-terminal Capeau cardiac cause Ce-emerin Ce-lamin Ce-MAN1 Cell Biol Cell Sci chromatin chromosome components cytoplasmic cytoskeleton de¢cient defects desmin di¡erent di¡erentiation dilated cardiomyopathy disease disrupt dynein e¡ect EDMD elegans embryos emerin Emery-Dreifuss muscular dystrophy encoding factor Fatkin FPLD function Gasser Gerace Goldman Gruenbaum HGPS Holaska human identi¢ed inner nuclear membrane integral proteins interaction intermediate lament Julien KASH domain lamin A/C gene laminopathies lipodystrophy LMNA LMNA mutations localization look mammalian MAN1 mechanical mitosis mitotic motor neuron mouse myocytes ndings nesprin normal nuclear envelope nuclear lamin nuclear lamina nuclear membrane nuclear membrane protein Nuclear organization nuclear pore complexes nucleoplasm nucleus overexpression partial lipodystrophy pathway patients peripherin phenotype progeria role signi¢cant skeletal muscle speci¢c splicing Starr Stewart structure syncoilin syndrome telomeres tissue transcription transfected transmembrane Werner’s Wilson KL Worman HJ yeast
Beliebte Passagen
Seite 15 - The p21 inhibitor of cyclin-dependent kinases controls DNA replication by interaction with PCNA.
Seite i - CIBA company of Basle, which merged with Sandoz in 1996, to form Novartis. The Foundation operates independently in London under English trust law. It was formally opened on 22 June 1949. The Foundation promotes the study and general knowledge of science and in particular encourages international co-operation in scientific research. To this end, it organizes internationally acclaimed meetings (typically eight symposia and allied open meetings and...
