Abstract：
:Meckel-Gruber syndrome (MKS) is a severe autosomal recessively inherited disorder caused by mutations in genes that encode components of the primary cilium and basal body. Here we show that two MKS proteins, MKS1 and meckelin, that are required for centrosome migration and ciliogenesis interact with actin-binding isoforms of nesprin-2 (nuclear envelope spectrin repeat protein 2, also known as Syne-2 and NUANCE). Nesprins are important scaffold proteins for maintenance of the actin cytoskeleton, nuclear positioning and nuclear-envelope architecture. However, in ciliated-cell models, meckelin and nesprin-2 isoforms colocalized at filopodia prior to the establishment of cell polarity and ciliogenesis. Loss of nesprin-2 and nesprin-1 shows that both mediate centrosome migration and are then essential for ciliogenesis, but do not otherwise affect apical-basal polarity. Loss of meckelin (by siRNA and in a patient cell-line) caused a dramatic remodelling of the actin cytoskeleton, aberrant localization of nesprin-2 isoforms to actin stress-fibres and activation of RhoA signalling. These findings further highlight the important roles of the nesprins during cellular and developmental processes, particularly in general organelle positioning, and suggest that a mechanistic link between centrosome positioning, cell polarity and the actin cytoskeleton is required for centrosomal migration and is essential for early ciliogenesis.
journal_name
J Cell Scijournal_title
Journal of cell scienceauthors
Dawe HR,Adams M,Wheway G,Szymanska K,Logan CV,Noegel AA,Gull K,Johnson CAdoi
10.1242/jcs.043794subject
Has Abstractpub_date
2009-08-01 00:00:00pages
2716-26issue
Pt 15eissn
0021-9533issn
1477-9137pii
jcs.043794journal_volume
122pub_type
杂志文章abstract：:Cdc6 and Cdt1 initiate DNA replication licensing when cells exit mitosis. In cycling cells, Cdc6 is efficiently degraded from anaphase onwards as a result of APC/C-Cdh1 activity. When APC/C-Cdh1 is switched off again, at the end of G1 phase, Cdc6 could thus re-accumulate, risking the re-licensing of DNA as long as Cdt...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.145862
更新日期：2014-03-15 00:00:00
abstract：:The extracellular signal-regulated protein kinase ERK1/2 is a crucial effector linking extracellular stimuli to cellular responses: upon phosphorylation ERK [also known as mitogen-activated protein kinase P42/P44 (MAPK)] concentrates in the nucleus where it activates specific programs of gene expression. Notwithstandi...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.03272
更新日期：2006-12-01 00:00:00
abstract：:The efficient functioning of striated muscle is dependent upon the structure of several cytoskeletal networks including myofibrils, microtubules, and intermediate filaments. However, little is known about how these networks function together during muscle differentiation and maintenance. In vitro studies suggest that ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.01158
更新日期：2004-07-01 00:00:00
abstract：:The control of bull spermatozoon flagellar activity has been investigated using direct current injection into the cells through an impaling glass microelectrode. Negative current injection results in a decrease in the flagellar frequency. Flagellar frequencies can be decreased to zero with high negative currents. This...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1979-02-01 00:00:00
abstract：:Histone proteins are essential for chromatin formation, and histone gene expression is coupled to DNA synthesis. In metazoans, the histone RNA binding protein HBP/SLBP is involved in post-transcriptional control of histone gene expression. In vitro assays have demonstrated that human HBP/SLBP is involved in histone mR...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.01523
更新日期：2004-12-01 00:00:00
abstract：:The post-translational modifications of the G protein of vesicular stomatitis virus, described in the preceding paper, indicate that its transport is arrested by carbonylcyanide m-chlorophenylhydrazone (CCCP) in or near the trans-Golgi. Immunofluorescence microscopy of BHK-21 cells infected with vesicular stomatitis v...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1989-04-01 00:00:00
abstract：:A comprehensive analysis of the molecular network of cellular factors establishing and maintaining pluripotency as well as self renewal of pluripotent stem cells is key for further progress in understanding basic stem cell biology. Nanog is necessary for the natural induction of pluripotency in early mammalian develop...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.167932
更新日期：2016-03-01 00:00:00
abstract：:Little is known about the molecular mechanisms that regulate the organization of vascular lumen. In this paper we show that lumen formation correlates with endothelial polarization. Adherens junctions (AJs) and VE-cadherin (VEC, encoded by CDH5) are required for endothelial apicobasal polarity in vitro and during embr...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.059329
更新日期：2010-04-01 00:00:00
abstract：:The ret finger protein (rfp) is a member of the B-box zinc finger gene family many of which may function in growth regulation and in the appropriate context become oncogenic. Members of this family are nuclear proteins that possess a characteristic tripartite motif consisting of the RING and B-box zinc binding domains...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1998-05-01 00:00:00
abstract：:SARA, an early endosomal protein, plays a key role in TGFβ signalling, as it presents SMAD2 and SMAD3 for phosphorylation by the activated TGFβ receptors. Here, we show that ERBIN is a new SARA-interacting protein that can be recruited by SARA to early endosomes. ERBIN was recently shown to bind and segregate phosphor...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.062307
更新日期：2011-10-01 00:00:00
abstract：:Polarity of many cell types is controlled by a protein complex consisting of Bazooka/PAR-3 (Baz), PAR-6 and atypical protein kinase C (aPKC). In Drosophila, the Baz-PAR-6-aPKC complex is required for the control of cell polarity in the follicular epithelium, in ectodermal epithelia and neuroblasts. aPKC is the main si...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.052514
更新日期：2009-10-15 00:00:00
abstract：:Phosphoinositides regulate numerous processes in various subcellular compartments. Whereas many stimuli trigger changes in the plasma-membrane PtdIns(4,5)P(2) concentration, little is known about its precursor, PtdIns(4)P, in particular whether there are stimulus-induced alterations independent of those of PtdIns(4,5)...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.060525
更新日期：2010-05-01 00:00:00
abstract：:Sorting endosomes (SEs) are the regulatory hubs for sorting cargo to multiple organelles, including lysosome-related organelles, such as melanosomes in melanocytes. In parallel, melanosome biogenesis is initiated from SEs with the processing and sequential transport of melanocyte-specific proteins toward maturing mela...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.216226
更新日期：2018-09-20 00:00:00
abstract：:A complex network of interactions between the stroma, the extracellular matrix and the epithelium drives mammary gland development and function. Two main assumptions in chemical carcinogenesis of the mammary gland have been that carcinogens induce neoplasia by causing mutations in the DNA of the epithelial cells and t...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.01000
更新日期：2004-03-15 00:00:00
abstract：:Microtubules--polymers of tubulin--perform essential functions, including regulation of cell shape, intracellular transport and cell motility. How microtubules are adapted to perform multiple diverse functions is not well understood. Post-translational modifications of tubulin subunits diversify the outer and luminal ...
journal_title：Journal of cell science
pub_type： 杂志文章,评审
doi：10.1242/jcs.063727
更新日期：2010-10-15 00:00:00
abstract：:We have used MPM-2, a monoclonal antibody raised against mitotic HeLa cells, to stain a Drosophila cell line, whole mounts of Drosophila embryos, and sectioned tissue from embryonic and larval stages of development. MPM-2 recognizes a major phosphoprotein of approximately 125 X 10(3) Mr in Drosophila tissue culture ce...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1987-02-01 00:00:00
abstract：:Epithelial cell polarity is essential for animal development. The scaffold protein Bazooka (Baz/PAR-3) forms apical polarity landmarks to organize epithelial cells. However, it is unclear how Baz is recruited to the plasma membrane and how this is coupled with downstream effects. Baz contains an oligomerization domain...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.091884
更新日期：2012-03-01 00:00:00
abstract：:The 16 kDa proteolipid (subunit c) of the eukaryotic vacuolar H(+)-ATPase (V-ATPase) is closely related to the ductin polypeptide that forms the connexon channel of gap junctions in the crustacean Nephrops norvegicus. Here we show that the major protein component of Manduca sexta gap junction preparations is a 16 kDa ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1994-07-01 00:00:00
abstract：:Cystogenesis is a morphological consequence of numerous genetic diseases of the epithelium. In the kidney, the pathogenic mechanisms underlying the program of altered cell and tubule morphology are obscured by secondary effects of cyst expansion. Here, we developed a new 3D tubuloid system to isolate the rapid changes...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.249557
更新日期：2020-07-16 00:00:00
abstract：:The karyo-cytoskeleton of cells cultured in vitro was investigated employing resinless section immunogold electron microscopy. Cells were entrapped in low-melting agarose, sequentially extracted with various buffers and digested with nucleases to obtain karyo-cytoskeletal frameworks and reacted with specific primary a...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1991-01-01 00:00:00
abstract：:Mutations in a gene (Klp38B) encoding a novel kinesin-like protein in Drosophila melanogaster lead to the formation of polyploid cells in the larval central nervous system and in the follicle cells of adult egg chambers. Some homozygous mutants survive to adulthood and also exhibit morphological defects indicative of ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1997-04-01 00:00:00
abstract：:The conversion of the cellular prion protein (PrPc) into a pathogenic isoform (PrP(Sc)) is one of the underlying events in the pathogenesis of the fatal transmissible spongiform encephalopathies (TSEs). Numerous compounds have been described to inhibit prion replication and PrP(Sc) accumulation in cell culture. Among ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.02609
更新日期：2005-11-01 00:00:00
abstract：:Mastigonemes are hair-like projections on the flagella of various kinds of lower eukaryotes. We obtained a monoclonal antibody (mAb-MAST1) to mastigonemes of Chlamydomonas reinhardtii, and found that it reacts with a single flagellar glycoprotein of about 230 kDa. Interestingly, immunofluorescence microscopy demonstra...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1996-01-01 00:00:00
abstract：:Alpha4 integrins are used by leukocytes and neural crest derivatives for adhesion and migration during embryogenesis, immune responses and tumour invasion. The pro-migratory activity of alpha4 integrin is mediated in part through the direct binding of the cytoplasmic domain to paxillin. Here, using intermolecular FRET...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.049130
更新日期：2009-05-15 00:00:00
abstract：:In many physiological contexts, intracellular reduction-oxidation (redox) conditions and the unfolded protein response (UPR) are important for the control of cell life and death decisions. UPR is triggered by the disruption of endoplasmic reticulum (ER) homeostasis, also known as ER stress. Depending on the duration a...
journal_title：Journal of cell science
pub_type： 杂志文章,评审
doi：10.1242/jcs.153643
更新日期：2014-09-01 00:00:00
abstract：:The weak spindle integrity checkpoint in Drosophila spermatocytes has revealed a novel function of the gamma-tubulin ring complex (gammaTuRC) in maintaining spindle bipolarity throughout meiosis. Bipolar and bi-astral spindles could form in Drosophila mutants for dd4, the gene encoding the 91 kDa subunit of gammaTuRC....
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.00295
更新日期：2003-03-01 00:00:00
abstract：:The intraerythrocytic developmental cycle of Plasmodium falciparum is completed with the release of up to 32 invasive daughter cells, the merozoites, into the blood stream. Before release, the final step of merozoite development is the assembly of the cortical pellicle, a multi-layered membrane structure. This unique ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.181230
更新日期：2016-02-15 00:00:00
abstract：:Efficient trafficking of the cystic fibrosis transmembrane conductance regulator (CFTR) to and from the cell surface is essential for maintaining channel density at the plasma membrane (PM) and ensuring proper physiological activity. The most common mutation, F508del, exhibits reduced surface expression and impaired f...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.120196
更新日期：2013-06-15 00:00:00
abstract：:Delayed wound healing caused by inefficient re-epithelialization underlines chronic skin lesions such as those found in diabetes. The dioxin receptor (AhR) modulates cell plasticity and migration and its activation by occupational polycyclic aromatic hydrocarbons (PAHs) results in severe skin lesions such as contact h...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.047274
更新日期：2009-06-01 00:00:00
abstract：:Understanding the interactions between distinct epithelial cells would help us to understand the development of tissues. Drosophila imaginal discs, which are made up of two types of epithelial cells, provide good model systems for such studies. The disc proper or the columnar epithelial cells are apposed to a layer of...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.02464
更新日期：2005-08-01 00:00:00
