abstract：:Nemaline myopathy (NM) is a congenital myopathy with an estimated incidence of 150,000 live births. It is caused by mutations in thin filament components, including nebulin, which accounts for about 50% of the cases. The identification of NM cases with nonsense mutations resulting in loss of the extreme C-terminal SH3...

journal_title：Journal of cell science

authors： Yamamoto DL,Vitiello C,Zhang J,Gokhin DS,Castaldi A,Coulis G,Piaser F,Filomena MC,Eggenhuizen PJ,Kunderfranco P,Camerini S,Takano K,Endo T,Crescenzi M,Luther PK,Lieber RL,Chen J,Bang ML

更新日期：2013-12-01 00:00:00

abstract：:Atherosclerosis depends on risk factors such as hyperlipidemia, smoking, hypertension and diabetes. Although these risk factors are relatively constant throughout the arterial circulation, atherosclerotic plaques occur at specific sites where flow patterns are disturbed, with lower overall magnitude and complex change...

journal_title：Journal of cell science

authors： Conway DE,Schwartz MA

更新日期：2013-11-15 00:00:00

abstract：:The ryanodine receptor (RyR) is an ion channel composed of four identical subunits mediating calcium efflux from the endo/sarcoplasmic reticulum of excitable and non-excitable cells. We present several lines of evidence indicating that the RyR2 N-terminus is capable of self-association. A combination of yeast two-hybr...

journal_title：Journal of cell science

authors： Zissimopoulos S,Viero C,Seidel M,Cumbes B,White J,Cheung I,Stewart R,Jeyakumar LH,Fleischer S,Mukherjee S,Thomas NL,Williams AJ,Lai FA

更新日期：2013-11-01 00:00:00

abstract：:Brain-derived neurotrophic factor (BDNF) is known to be a crucial regulator of neuronal survival and synaptic plasticity in the mammalian brain. Furthermore, BDNF positively influences differentiation of embryonic neural precursors, as well as that of neural stem cells from adult neurogenic niches. To study the impact...

journal_title：Journal of cell science

authors： Leschik J,Eckenstaler R,Nieweg K,Lichtenecker P,Brigadski T,Gottmann K,Lessmann V,Lutz B

更新日期：2013-11-01 00:00:00

abstract：:The mechanisms by which volatile general anaesthetics (VAs) produce a depression of central nervous system are beginning to be better understood, but little is known about a number of side effects. Here, we show that the cold receptor transient receptor potential melastatin 8 (TRPM8) undergoes a complex modulation by ...

journal_title：Journal of cell science

authors： Vanden Abeele F,Kondratskyi A,Dubois C,Shapovalov G,Gkika D,Busserolles J,Shuba Y,Skryma R,Prevarskaya N

更新日期：2013-10-01 00:00:00

abstract：:Mechanical stimulation affects many biological aspects in living cells through mechanotransduction. In myogenic precursor cells (MPCs), mechanical stimulation activates p38 mitogen-activated protein kinase (MAPK), a key regulator of myogenesis, via activating TNFα-converting enzyme (TACE, also known as ADAM17), to rel...

journal_title：Journal of cell science

authors： Niu A,Wen Y,Liu H,Zhan M,Jin B,Li YP

更新日期：2013-10-01 00:00:00

abstract：:Epidermal growth factor receptor (EGFR) ligands undergo a complex series of processing events during their maturation to active signaling proteins. Like its mammalian homologs, the predominant Drosophila EGFR ligand Spitz is produced as a transmembrane pro-protein. In the secretory pathway, Spitz is cleaved within its...

journal_title：Journal of cell science

authors： Steinhauer J,Liu HH,Miller E,Treisman JE

更新日期：2013-10-01 00:00:00

abstract：:Notch signaling is a conserved signaling pathway implicated in embryogenesis and adult tissue maintenance. Notch signaling strength is strictly regulated, notably by maintaining a controlled pool of functional receptor at the cell surface. Mammalian non-activated Notch receptor is internalized, ubiquitylated by the It...

journal_title：Journal of cell science

authors： Puca L,Chastagner P,Meas-Yedid V,Israël A,Brou C

更新日期：2013-10-01 00:00:00

abstract：:The hedgehog (Hh) signaling pathway plays a very important role in metazoan development by controlling pattern formation. Malfunction of the Hh signaling pathway leads to numerous serious human diseases, including congenital disorders and cancers. The seven-transmembrane domain protein Smoothened (Smo) is a key transd...

journal_title：Journal of cell science

authors： Yang X,Mao F,Lv X,Zhang Z,Fu L,Lu Y,Wu W,Zhou Z,Zhang L,Zhao Y

更新日期：2013-09-15 00:00:00

abstract：:Cell migration makes a fundamental contribution to both normal physiology and disease pathogenesis. Integrin engagement with extracellular ligands spatially controls, via the cyclical activation and deactivation of the small GTPase Rac1, the dynamic membrane protrusion and cytoskeletal reorganization events that are r...

journal_title：Journal of cell science

authors： Jacquemet G,Morgan MR,Byron A,Humphries JD,Choi CK,Chen CS,Caswell PT,Humphries MJ

更新日期：2013-09-15 00:00:00

abstract：:Phosphatidylinositol phosphates are important regulators of processes such as the cytoskeleton organization, membrane trafficking and gene transcription, which are all crucial for polarized cell growth. In particular, phosphatidylinositol 4,5-bisphosphate [PtdIns(4,5)P2] has essential roles in polarized growth as well...

journal_title：Journal of cell science

authors： Guillas I,Vernay A,Vitagliano JJ,Arkowitz RA

更新日期：2013-08-15 00:00:00

abstract：:Prion diseases are fatal neurodegenerative diseases characterised by the accumulation of misfolded prion protein (PrP(Sc)) in the brain. They are caused by the templated misfolding of normal cellular protein, PrP(C), by PrP(Sc). We have recently generated a unique cell system in which epitope-tagged PrP(C) competent t...

journal_title：Journal of cell science

authors： Goold R,McKinnon C,Rabbanian S,Collinge J,Schiavo G,Tabrizi SJ

更新日期：2013-08-15 00:00:00

abstract：:Super-resolution fluorescence imaging by single-molecule photoactivation or photoswitching and position determination (localization microscopy) has the potential to fundamentally revolutionize our understanding of how cellular function is encoded at the molecular level. Among all powerful, high-resolution imaging tech...

journal_title：Journal of cell science

authors： Sauer M

更新日期：2013-08-15 00:00:00

abstract：:Regulation of cell-cell contacts is essential for integrity of the vascular endothelium. Here, a critical role of the F-actin-binding protein drebrin in maintaining endothelial integrity is revealed under conditions mimicking vascular flow. Drebrin knockdown leads to weakening of cell-cell contacts, characterized by l...

journal_title：Journal of cell science

authors： Rehm K,Panzer L,van Vliet V,Genot E,Linder S

更新日期：2013-08-15 00:00:00

abstract：:Neural stem cells (NSCs) typically show efficient self-renewal and selective differentiation. Their invasion potential, however, is not well studied. In this study, Sox2-positive NSCs from the E14.5 rat cortex were found to be non-invasive and showed only limited migration in vitro. By contrast, FGF2-expanded NSCs sho...

journal_title：Journal of cell science

authors： Sailer MH,Gerber A,Tostado C,Hutter G,Cordier D,Mariani L,Ritz MF

更新日期：2013-08-15 00:00:00

abstract：:Mitotic spindle assembly in animal cells is orchestrated by a chromosome-dependent pathway that directs microtubule stabilization. RanGTP generated at chromosomes releases spindle assembly factors from inhibitory complexes with importins, the nuclear transport factors that facilitate protein import into the nucleus du...

journal_title：Journal of cell science

authors： Wu Z,Jiang Q,Clarke PR,Zhang C

更新日期：2013-08-01 00:00:00

abstract：:Protrusion formation is the first step that precedes cell movement of motile cells. Spatial control of actin polymerization is necessary to achieve directional protrusion during cell migration. Here we show that the spatial coordinators p190RhoGEF and p190RhoGAP regulate actin polymerization during leading edge protru...

journal_title：Journal of cell science

authors： Bravo-Cordero JJ,Sharma VP,Roh-Johnson M,Chen X,Eddy R,Condeelis J,Hodgson L

更新日期：2013-08-01 00:00:00

abstract：:Triosephosphate isomerase (TPI) is a glycolytic enzyme that converts dihydroxyacetone phosphate (DHAP) into glyceraldehyde 3-phosphate (GAP). Glycolytic enzyme dysfunction leads to metabolic diseases collectively known as glycolytic enzymopathies. Of these enzymopathies, TPI deficiency is unique in the severity of neu...

journal_title：Journal of cell science

authors： Roland BP,Stuchul KA,Larsen SB,Amrich CG,Vandemark AP,Celotto AM,Palladino MJ

更新日期：2013-07-15 00:00:00

abstract：:In sciarid flies (Diptera, Sciaridae), one or two paternally derived X chromosomes are discarded from the soma at early cleavages to determine the sex of the embryo (XX, females; X0, males). X chromosome(s) elimination is achieved by an abnormal anaphase segregation so that X sister chromatids do not reach the poles a...

journal_title：Journal of cell science

authors： Escribá MC,Goday C

更新日期：2013-07-15 00:00:00

abstract：:Actomyosin stress fibers (SFs) enable cells to exert traction on planar extracellular matrices (ECMs) by tensing focal adhesions (FAs) at the cell-ECM interface. Although it is widely appreciated that the spatial and temporal distribution of these tensile forces play key roles in polarity, motility, fate choice, and o...

journal_title：Journal of cell science

authors： Chang CW,Kumar S

更新日期：2013-07-15 00:00:00

abstract：:Wnts are glycan- and lipid-modified morphogens that are important for cellular responses, but how Wnts are secreted in polarized epithelial cells remains unclear. Although Wntless (Wls) has been shown to interact with Wnts and support their secretion, the role of Wls in the sorting of Wnts to the final destination in ...

journal_title：Journal of cell science

authors： Yamamoto H,Awada C,Hanaki H,Sakane H,Tsujimoto I,Takahashi Y,Takao T,Kikuchi A

更新日期：2013-07-01 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

authors： Holleran JP,Zeng J,Frizzell RA,Watkins SC

更新日期：2013-06-15 00:00:00

abstract：:The coordinated trafficking and tethering of membrane cargo within cells relies on the function of distinct cytoskeletal motors that are targeted to specific subcellular compartments through interactions with protein adaptors and phospholipids. The unique actin motor myosin VI functions at distinct steps during clathr...

journal_title：Journal of cell science

authors： Tumbarello DA,Kendrick-Jones J,Buss F

更新日期：2013-06-15 00:00:00

abstract：:In eukaryotes, mRNA export involves many evolutionarily conserved factors that carry the nascent transcript to the nuclear pore complex (NPC). The THO/TREX complex couples transcription to mRNA export and recruits the mRNA export receptor NXF1 for the transport of messenger ribonucleoprotein particles (mRNP) to the NP...

journal_title：Journal of cell science

authors： Umlauf D,Bonnet J,Waharte F,Fournier M,Stierle M,Fischer B,Brino L,Devys D,Tora L

更新日期：2013-06-15 00:00:00

abstract：:Nonsense-mediated mRNA decay (NMD) is an evolutionarily conserved mRNA surveillance system that degrades mRNA transcripts that harbour a premature translation-termination codon (PTC), thus reducing the synthesis of truncated proteins that would otherwise have deleterious effects. Although extensive research has identi...

journal_title：Journal of cell science

authors： Fang Y,Bateman JF,Mercer JF,Lamandé SR

更新日期：2013-06-15 00:00:00

abstract：:The asparaginyl hydroxylase factor inhibiting HIF-1 (FIH-1) is an important suppressor of hypoxia-inducible factor (HIF) activity. In addition to HIF-α, FIH-1 was previously shown to hydroxylate other substrates within a highly conserved protein interaction domain, termed the ankyrin repeat domain (ARD). However, to d...

journal_title：Journal of cell science

authors： Janke K,Brockmeier U,Kuhlmann K,Eisenacher M,Nolde J,Meyer HE,Mairbäurl H,Metzen E

更新日期：2013-06-15 00:00:00

abstract：:Transcription factor IIH (TFIIH) participates in transcription, nucleotide excision repair and the control of the cell cycle. In the present study, we demonstrate that the Dmp52 subunit of TFIIH in Drosophila physically interacts with the fly p53 homologue, Dp53. The depletion of Dmp52 in the wing disc generates chrom...

journal_title：Journal of cell science

authors： Villicaña C,Cruz G,Zurita M

更新日期：2013-06-01 00:00:00

abstract：:Regulated cell shape changes in epithelial cells, which contribute to most organs and tissues, are at the basis of morphogenesis. Crumbs (Crb) is an essential apical determinant controlling epithelial apicobasal polarity. Here we provide evidence for a novel role of Crb apical localisation and stabilisation in control...

journal_title：Journal of cell science

authors： Letizia A,Ricardo S,Moussian B,Martín N,Llimargas M

更新日期：2013-05-15 00:00:00

abstract：:The conventional kinesin motor transports many different cargos to specific locations in neurons. How cargos regulate motor function remains unclear. Here we focus on KIF5, the heavy chain of conventional kinesin, and report that the Kv3 (Shaw) voltage-gated K(+) channel, the only known tetrameric KIF5-binding protein...

journal_title：Journal of cell science

authors： Barry J,Xu M,Gu Y,Dangel AW,Jukkola P,Shrestha C,Gu C

更新日期：2013-05-01 00:00:00

abstract：:Protein with tau-like repeats (PTL-1) is the sole Caenorhabditis elegans homolog of tau and MAP2, which are members of the mammalian family of microtubule-associated proteins (MAPs). In mammalian neurons, tau and MAP2 are segregated, with tau being mainly localised to the axon and MAP2 mainly to the dendrite. In parti...

journal_title：Journal of cell science

authors： Chew YL,Fan X,Götz J,Nicholas HR

更新日期：2013-05-01 00:00:00

abstract：:Loss of tissue organization is a hallmark of the early stages of cancer, and there is considerable interest in proteins that maintain normal tissue architecture. Prostate epithelial cells cultured in Matrigel form three-dimensional acini that mimic aspects of prostate gland development. The organization of these struc...

journal_title：Journal of cell science

authors： Romero D,Kawano Y,Bengoa N,Walker MM,Maltry N,Niehrs C,Waxman J,Kypta R

更新日期：2013-04-15 00:00:00

abstract：:Patients with myotonic dystrophy type 1 exhibit a diversity of symptoms that affect many different organs. Among these are cognitive dysfunctions, the origin of which has remained elusive, partly because of the difficulty in accessing neural cells. Here, we have taken advantage of pluripotent stem cell lines derived f...

journal_title：Journal of cell science

authors： Denis JA,Gauthier M,Rachdi L,Aubert S,Giraud-Triboult K,Poydenot P,Benchoua A,Champon B,Maury Y,Baldeschi C,Scharfmann R,Piétu G,Peschanski M,Martinat C

更新日期：2013-04-15 00:00:00

abstract：:Polarization of early embryos provides a foundation to execute essential patterning and morphogenetic events. In Caenorhabditis elegans, cell contacts polarize early embryos along their radial axis by excluding the cortical polarity protein PAR-6 from sites of cell contact, thereby restricting PAR-6 to contact-free ce...

journal_title：Journal of cell science

authors： Chan E,Nance J

更新日期：2013-04-01 00:00:00

abstract：:DNA replication in eukaryotic cells initiates from multiple replication origins that are distributed throughout the genome. Coordinating the usage of these origins is crucial to ensure complete and timely replication of the entire genome precisely once in each cell cycle. Replication origins fire according to a cell-t...

journal_title：Journal of cell science

authors： Yekezare M,Gómez-González B,Diffley JF

更新日期：2013-03-15 00:00:00

abstract：:Abnormal cardiac valve morphogenesis is a common cause of human congenital heart disease. The molecular mechanisms regulating endocardial cell proliferation and differentiation into cardiac valves remain largely unknown, although great progress has been made on the endocardial contribution to the atrioventricular cush...

journal_title：Journal of cell science

authors： Wang X,Yu Q,Wu Q,Bu Y,Chang NN,Yan S,Zhou XH,Zhu X,Xiong JW

更新日期：2013-03-15 00:00:00

abstract：:MicroRNAs are known to play regulatory roles in gene expression associated with cancer development. We analyzed levels of the microRNA miR-24 in patients with breast carcinoma and found that miR-24 was higher in breast carcinoma samples than in benign breast tissues. We generated constructs expressing miR-24 and studi...

journal_title：Journal of cell science

authors： Du WW,Fang L,Li M,Yang X,Liang Y,Peng C,Qian W,O'Malley YQ,Askeland RW,Sugg SL,Qian J,Lin J,Jiang Z,Yee AJ,Sefton M,Deng Z,Shan SW,Wang CH,Yang BB

更新日期：2013-03-15 00:00:00

abstract：:The roles of P21-activated kinase (PAK) in the regulation of axon outgrowth downstream of extracellular matrix (ECM) proteins are poorly understood. Here we show that PAK1-3 and PIX are expressed in the developing spinal cord and differentially localize to point contacts and filopodial tips within motile growth cones....

journal_title：Journal of cell science

authors： Santiago-Medina M,Gregus KA,Gomez TM

更新日期：2013-03-01 00:00:00

abstract：:Both at a basal level and after induction (especially in response to nutrient starvation), the function of autophagy is to allow cells to degrade and recycle damaged organelles, proteins and other biological constituents. Here, we focus on the role microtubules have in autophagosome formation, autophagosome transport ...

journal_title：Journal of cell science

authors： Mackeh R,Perdiz D,Lorin S,Codogno P,Poüs C

更新日期：2013-03-01 00:00:00

abstract：:Endothelial cells (ECs) sense shear stress and transduce blood flow information into functional responses that play important roles in vascular homeostasis and pathophysiology. A unique feature of shear-stress-sensing is the involvement of many different types of membrane-bound molecules, including receptors, ion chan...

journal_title：Journal of cell science

authors： Yamamoto K,Ando J

更新日期：2013-03-01 00:00:00

abstract：:Occludin plays an important role in maintaining tight junction barrier function in many types of epithelia. We previously reported that activation of transient receptor potential vanilloid subtype 1 (TRPV1) in rabbit submandibular gland promoted salivary secretion, partly by an increase in paracellular permeability. W...

journal_title：Journal of cell science

authors： Cong X,Zhang Y,Yang NY,Li J,Ding C,Ding QW,Su YC,Mei M,Guo XH,Wu LL,Yu GY

更新日期：2013-03-01 00:00:00