LEADER 04126nam 22006374a 450 001 9910841497503321 005 20230617040502.0 010 $a1-280-27638-X 010 $a9786610276387 010 $a0-470-09376-5 010 $a0-470-09374-9 035 $a(CKB)1000000000356526 035 $a(EBL)239056 035 $a(SSID)ssj0000212675 035 $a(PQKBManifestationID)11184580 035 $a(PQKBTitleCode)TC0000212675 035 $a(PQKBWorkID)10138376 035 $a(PQKB)10193159 035 $a(MiAaPQ)EBC239056 035 $a(OCoLC)85820611 035 $a(EXLCZ)991000000000356526 100 $a20041104d2005 uy 0 101 0 $aeng 135 $aur|n|---||||| 181 $ctxt 182 $cc 183 $acr 200 10$aNuclear organization in development and disease$b[electronic resource] /$f[editors, Derek Chadwick, Jamie Goode] 210 $aHoboken, NJ $cWiley$d2005 215 $a1 online resource (302 p.) 225 1 $aNovartis Foundation symposium ;$v264 300 $aDescription based upon print version of record. 311 $a0-470-09373-0 320 $aIncludes bibliographical references and index. 327 $aNUCLEAR ORGANIZATION IN DEVELOPMENT AND DISEASE; Contents; Participants; Chair's introduction; Nuclear lamins: building blocks of nuclear structure and function; Discussion; Aspects of nuclear envelope dynamics in mitotic cells; Discussion; Components of the nuclear envelope and their role in human disease; Discussion; Nuclear membrane protein emerin: roles in gene regulation, actin dynamics and human disease; Discussion; Identification of novel integral membrane proteins of the nuclear envelope with potential disease links using subtractive proteomics; Discussion; Genetics of laminopathies 327 $aDiscussionMuscular dystrophies related to the cytoskeleton/nuclear envelope; Discussion; Skeletal and cardiac muscle defects in a murine model of Emery-Dreifuss muscular dystrophy; Discussion; Multiple pathways tether telomeres and silent chromatin at the nuclear periphery: functional implications for Sir-mediated repression; Discussion; A-type lamin-linked lipodystrophies; Discussion; Cytoskeletal defects in amyotrophic lateral sclerosis (motor neuron disease); Discussion; LMNA mutations in progeroid syndromes; Discussion 327 $aA genetic approach to study the role of nuclear envelope components in nuclear positioningDiscussion; General discussion I; A lamin-dependent pathway that regulates nuclear organization, cell-cycle progression and germ-cell development; Discussion; Mutations in the mouse Lmna gene causing progeria, muscular dystrophy and cardiomyopathy; Discussion; The nuclear membrane and mechanotransduction: impaired nuclear mechanics and mechanotransduction in lamin A/C-deficient cells; Discussion; Chair's summing-up; Index of contributors; Subject index 330 $aThis book draws together contributions from cell and developmental biologists, structural biologists, geneticists and clinical scientists aimed at a better understanding of the cellular and molecular basis of these diseases. Topics include:How nuclear structure and location within a nucleus affect gene expressionChromatin organization and cell differentiationThe nature of the interactions between the nuclear envelope and the cytoskeletonThe extent to which the cytoskeleton mediates communication between the cell membrane and nucleus in regulating gene expression and whether 410 0$aNovartis Foundation symposium ;$v264. 606 $aCell nuclei$vCongresses 606 $aCell nuclei$xAbnormalities$vCongresses 606 $aDevelopmental cytology$vCongresses 615 0$aCell nuclei 615 0$aCell nuclei$xAbnormalities 615 0$aDevelopmental cytology 676 $a571.936 676 $a571.9366 700 $aChadwick$b Derek$091632 701 $aGoode$b Jamie$0283336 801 0$bMiAaPQ 801 1$bMiAaPQ 801 2$bMiAaPQ 906 $aBOOK 912 $a9910841497503321 996 $aNuclear organization in development and disease$94140837 997 $aUNINA