Abstract：
:Transcripts containing expanded CNG repeats, which are found in several neuromuscular diseases, are not exported from the nucleus and aggregate as ribonuclear inclusions by an unknown mechanism. Using the MS2-GFP system, which tethers fluorescent proteins to a specific mRNA, we followed the dynamics of single CUG-repeat transcripts and RNA aggregation in living cells. Single transcripts with 145 CUG repeats from the dystrophia myotonica-protein kinase (DMPK) gene had reduced diffusion kinetics compared with transcripts containing only five CUG repeats. Fluorescence recovery after photobleaching (FRAP) experiments showed that CUG-repeat RNAs display a stochastic aggregation behaviour, because individual RNA foci formed at different rates and displayed different recoveries. Spontaneous clustering of CUG-repeat RNAs was also observed, confirming the stochastic aggregation revealed by FRAP. The splicing factor Mbnl1 colocalized with individual CUG-repeat transcripts and its aggregation with RNA foci displayed the same stochastic behaviour as CUG-repeat mRNAs. Moreover, depletion of Mbnl1 by RNAi resulted in decreased aggregation of CUG-repeat transcripts after FRAP, supporting a direct role for Mbnl1 in CUG-rich RNA foci formation. Our data reveal that nuclear CUG-repeat RNA aggregates are labile, constantly forming and disaggregating structures, and that the Mbnl1 splicing factor is directly involved in the aggregation process.
journal_name
J Cell Scijournal_title
Journal of cell scienceauthors
Querido E,Gallardo F,Beaudoin M,Ménard C,Chartrand Pdoi
10.1242/jcs.073270subject
Has Abstractpub_date
2011-05-15 00:00:00pages
1703-14issue
Pt 10eissn
0021-9533issn
1477-9137pii
jcs.073270journal_volume
124pub_type
杂志文章abstract：:Eggs of Urechis caupo are surrounded by a congruent to 0.9 micrometer thick egg envelope and, attached to that, a peripheral jelly layer about 3 micrometers thick. Before fertilization, the sperm undergoes the acrosome reaction and binds to the egg envelope. As part of a study of the induction of the acrosome reaction...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1985-03-01 00:00:00
abstract：:A conjugate of horseradish peroxidase (HRP) to poly(L-lysine) (PLL) was used as a non-specific adsorptive probe to study transcytosis in MDCK strain I and Caco-2 epithelial cells. As we have shown previously, HRP-PLL transcytosis proceeds via an intracellular, non-lysosomal proteolytic compartment in MDCK cells; yet, ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1993-12-01 00:00:00
abstract：:Septins have been shown to play important roles in cytokinesis in diverse organisms ranging from yeast to mammals. In this study, we show that both the unc-59 and unc-61 loci encode Caenorhabditis elegans septins. Genomic database searches indicate that unc-59 and unc-61 are probably the only septin genes in the C. el...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：2000-11-01 00:00:00
abstract：:There is a limited number of methods to examine transbilayer lipid distribution in biomembranes. We employed freeze-fracture replica-labelling immunoelectron microscopy in combination with lipid-binding proteins and a peptide to examine both transbilayer distribution and lateral distribution of various phospholipids i...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.163105
更新日期：2015-04-15 00:00:00
abstract：:In many cells, the endoplasmic reticulum (ER) contains segregated smooth and rough domains, but the mechanism of this segregation is unclear. Here, we used a HeLa cell line, inducibly expressing a GFP fusion protein [GFP-b(5)tail] anchored to the ER membrane, as a tool to investigate factors influencing ER organisatio...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.03058
更新日期：2006-08-01 00:00:00
abstract：:Centaurin-alpha(1) is a phosphatidylinositol 3,4,5-trisphosphate binding protein as well as a GTPase activating protein (GAP) for the ADP-ribosylation factor (ARF) family of small GTPases. To further understand its cellular function, we screened a rat brain cDNA library using centaurin-alpha(1) as bait to identify cen...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.02369
更新日期：2005-06-01 00:00:00
abstract：:Rab GTPases regulate various membrane trafficking pathways but the mechanisms by which GTPase-activating proteins recognise specific Rabs are not clear. Rab8 is involved in controlling several trafficking processes, including the trafficking of transferrin receptor from the early endosome to the recycling endosome. He...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.102327
更新日期：2012-11-01 00:00:00
abstract：:We investigated the role of Pav-KLP, a kinesin-6, in the coordination of spindle and cortical dynamics during mitosis in Drosophila embryos. In vitro, Pav-KLP behaves as a dimer. In vivo, it localizes to mitotic spindles and furrows. Inhibition of Pav-KLP causes defects in both spindle dynamics and furrow ingression, ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.064048
更新日期：2010-06-01 00:00:00
abstract：:Two monoclonal antibodies, BA16 and BA17, have been developed using a detergent-insoluble extract of human mammary epithelial organoids as immunogen. Indirect immunofluorescent staining of cultured cells showed that the component reacting with the antibodies was filamentous and the intensity of staining was stronger i...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1985-04-01 00:00:00
abstract：:Formins constitute a family of eukaryotic proteins that are considered to function as a cytoskeleton organizer to regulate morphogenesis, cell polarity and cytokinesis. Fhos is a recently identified mammalian formin, which contains the conserved domains FH (formin homology) 1 and FH2 in the middle region and the Dia-a...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.00769
更新日期：2003-11-15 00:00:00
abstract：:Cofilin has emerged as a key regulator of actin dynamics at the leading edge of motile cells. Through its actin-severing activity, it creates new actin barbed ends for polymerization and also depolymerizes old actin filaments. Its function is tightly regulated in the cell. Spatially, its activity is restricted by othe...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.01631
更新日期：2005-01-01 00:00:00
abstract：:When migrating mesenchymal cells collide, they exhibit a 'contact inhibition of locomotion' response that results in reversal of their front-rear polarity by extension of a new leading edge, which enables their migration away from the opposing contacted cell. The critical cytoskeletal rearrangements underpinning these...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.087965
更新日期：2011-08-01 00:00:00
abstract：:Double minutes (DMs) seen in a substantial fraction of human tumors are the cytogenetic manifestation of gene amplification which renders the tumor cells advantageous for growth and survival. DMs are acentric and atelomeric chromatin composed of circular DNA. In this study, we found they showed a remarkable relocation...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1998-11-01 00:00:00
abstract：:Cell migration involves the dynamic formation and release of cell-substrate adhesions, where the exertion and detection of mechanical forces take place. Members of the calpain family of calcium-dependent proteases are believed to have a central role in these processes, possibly through the regulation of focal adhesion...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.036152
更新日期：2008-11-01 00:00:00
abstract：:Mesenchymal stem cells (MSCs) are among the most promising and suitable stem cell types for vascular tissue engineering. Substantial effort has been made to differentiate MSCs towards vascular cell phenotypes, including endothelial cells and smooth muscle cells (SMCs). The microenvironment of vascular cells not only c...
journal_title：Journal of cell science
pub_type： 杂志文章,评审
doi：10.1242/jcs.167783
更新日期：2015-07-15 00:00:00
abstract：:Kindlin-2 is a FERM and PH domain-containing integrin-binding protein that is emerging as an important regulator of integrin activation. How kindlin-2 functions in integrin activation, however, is not known. We report here that kindlin-2 interacts with multiple phosphoinositides, preferentially with phosphatidylinosit...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.076976
更新日期：2011-03-15 00:00:00
abstract：:The repair of injured tissue must occur rapidly to prevent microbial invasion and maintain tissue integrity. Epithelial tissues in particular, which serve as a barrier against the external environment, must repair efficiently in order to restore their primary function. Here we analyze the effect of different parameter...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.109066
更新日期：2012-12-15 00:00:00
abstract：:Cadherins are transmembrane glycoproteins involved in Ca2+-dependent cell-cell adhesion. Using L cells expressing one of three functional E-cadherin constructs, the wild-type, a chimeric molecule with alpha-catenin (EalphaC), and a tail-less one, we determined the effect of v-Src expression on E-cadherin-mediated adhe...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：2001-02-01 00:00:00
abstract：:Using a genome-wide technical knockout, we isolated a newly identified set of GRIM (genes associated with retinoid-interferon-induced mortality) genes; GRIM genes mediate IFN- and retinoic-acid (RA)-induced cell death. Here, we describe the isolation and characterization of one such gene, GRIM-1. Three proteins, with ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.070250
更新日期：2010-08-15 00:00:00
abstract：:The outer nuclear membrane proteins nesprin-1 and nesprin-2 are retained at the nuclear envelope through an interaction of their klarsicht/ANC-1/syne homology (KASH) domain with Sun proteins present at the inner nuclear membrane. We investigated the requirements for the localization of nesprin-3alpha at the outer nucl...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.014191
更新日期：2007-10-01 00:00:00
abstract：:The subnuclear organization of nucleic acids and cognate regulatory factors suggests that there are functional interrelationships between nuclear structure and gene expression. Nuclear proteins that are localized in discrete domains within the nucleus include the leukemia-associated acute myelogenous leukemia (AML) an...
journal_title：Journal of cell science
pub_type： 杂志文章,评审
doi：
更新日期：2000-07-01 00:00:00
abstract：:We have isolated mini-titin from the nematodes Ascaris lumbricoides and Caenorhabditis elegans under native conditions using a modification in the procedure to prepare this protein from insect muscle. The proteins have an apparent molecular weight of 600,000 and appear in oriented specimens as flexible thin rods with ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1991-04-01 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
pub_type： 杂志文章
doi：10.1242/jcs.119388
更新日期：2013-04-15 00:00:00
abstract：:The integrin beta1 cytoplasmic domain plays a key role in a variety of integrin-mediated events including adhesion, migration and signaling. A number of studies suggest that phosphorylation may modify the functional state of the cytoplasmic domain, but these studies frequently only examine the effect of substituting a...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：2001-07-01 00:00:00
abstract：:When oxidative phosphorylation is inhibited in cells of Dictyostelium discoideum, the phosphorylation of tyrosine residues on actin is strongly increased. This increase is fully reversible. Under the same conditions the amoeboid cells undergo a series of shape changes. Within three minutes the pseudopods are withdrawn...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1994-01-01 00:00:00
abstract：:Cellular lipids play crucial roles in the cell, including in energy storage, the formation of cellular membranes, and in signaling and vesicular trafficking. To understand the functions and characteristics of lipids within cells, various methods to image lipids have been established. In this Commentary, we discuss the...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.150524
更新日期：2014-11-15 00:00:00
abstract：:Electrical synapses (gap junctions) rapidly transmit signals between neurons and are composed of connexins. In neurons, connexin36 (Cx36) is the most abundant isoform; however, the mechanisms underlying formation of Cx36-containing electrical synapses are unknown. We focus on homocellular and heterocellular gap juncti...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.133066
更新日期：2014-03-15 00:00:00
abstract：:Directed axonal growth depends on correct coordination of the actin and microtubule cytoskeleton in the growth cone. However, despite the relatively large number of proteins implicated in actin-microtubule crosstalk, the mechanisms whereby actin polymerization is coupled to microtubule stabilization and advancement in...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.203455
更新日期：2017-08-01 00:00:00
abstract：:Collapsin response mediator proteins (CRMPs) mediate signal transduction of neurite outgrowth and axonal guidance during neuronal development. Voltage-gated Ca(2+) channels and interacting proteins are essential in neuronal signaling and synaptic transmission during this period. We recently identified the presynaptic ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.053280
更新日期：2009-12-01 00:00:00
abstract：:BMP2 stimulates bone formation and signals preferably through BMP receptor (BMPR) 1A, whereas GDF5 is a cartilage inducer and signals preferably through BMPR1B. Consequently, BMPR1A and BMPR1B are believed to be involved in bone and cartilage formation, respectively. However, their function is not yet fully clarified....
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.246934
更新日期：2020-08-21 00:00:00
