Partial restoration of mutant enzyme homeostasis in three distinct lysosomal storage disease cell lines by altering calcium homeostasis.

abstract：:Signaling of the cytokine interleukin-6 (IL-6) via its soluble IL-6 receptor (sIL-6R) is responsible for the proinflammatory properties of IL-6 and constitutes an attractive therapeutic target, but how the sIL-6R is generated in vivo remains largely unclear. Here, we use liquid chromatography-mass spectrometry to iden...

journal_title：PLoS biology

authors： Riethmueller S,Somasundaram P,Ehlers JC,Hung CW,Flynn CM,Lokau J,Agthe M,Düsterhöft S,Zhu Y,Grötzinger J,Lorenzen I,Koudelka T,Yamamoto K,Pickhinke U,Wichert R,Becker-Pauly C,Rädisch M,Albrecht A,Hessefort M,Stahnke

更新日期：2017-01-06 00:00:00

abstract：:The ability to use stored information in a highly flexible manner is a defining feature of the declarative memory system. However, the neuronal mechanisms underlying this flexibility are poorly understood. To address this question, we recorded single-unit activity from the hippocampus of 2 nonhuman primates performing...

journal_title：PLoS biology

authors： Yang C,Naya Y

更新日期：2020-11-18 00:00:00

abstract：:The disc-large (DLG)-membrane-associated guanylate kinase (MAGUK) family of proteins forms a central signaling hub of the glutamate receptor complex. Among this family, some proteins regulate developmental maturation of glutamatergic synapses, a process vulnerable to aberrations, which may lead to neurodevelopmental d...

journal_title：PLoS biology

authors： Favaro PD,Huang X,Hosang L,Stodieck S,Cui L,Liu YZ,Engelhardt KA,Schmitz F,Dong Y,Löwel S,Schlüter OM

更新日期：2018-12-26 00:00:00

abstract：:Eukaryotic protein kinases regulate most cellular functions by phosphorylating targeted protein substrates through a highly conserved catalytic core. In the active state, the catalytic core oscillates between open, intermediate, and closed conformations. Currently, the intramolecular interactions that regulate the act...

journal_title：PLoS biology

authors： Meharena HS,Fan X,Ahuja LG,Keshwani MM,McClendon CL,Chen AM,Adams JA,Taylor SS

更新日期：2016-11-30 00:00:00

abstract：:In their competition for hosts, parasites with antigens that are novel to the host immune system will be at a competitive advantage. The resulting frequency-dependent selection can structure parasite populations into strains of limited genetic overlap. For the causative agent of malaria, Plasmodium falciparum, the hig...

journal_title：PLoS biology

authors： Pilosof S,He Q,Tiedje KE,Ruybal-Pesántez S,Day KP,Pascual M

更新日期：2019-06-24 00:00:00

abstract：:Differentiated T helper (Th) cell lineages are thought to emerge from alternative cell fate decisions. However, recent studies indicated that differentiated Th cells can adopt mixed phenotypes during secondary immunological challenges. Here we show that natural primary immune responses against parasites generate bifun...

journal_title：PLoS biology

authors： Peine M,Rausch S,Helmstetter C,Fröhlich A,Hegazy AN,Kühl AA,Grevelding CG,Höfer T,Hartmann S,Löhning M

更新日期：2013-01-01 00:00:00

abstract：:Our current understanding of biology is heavily based on a small number of genetically tractable model organisms. Most eukaryotic phyla lack such experimental models, and this limits our ability to explore the molecular mechanisms that ultimately define their biology, ecology, and diversity. In particular, marine prot...

journal_title：PLoS biology

authors： Waller RF,Cleves PA,Rubio-Brotons M,Woods A,Bender SJ,Edgcomb V,Gann ER,Jones AC,Teytelman L,von Dassow P,Wilhelm SW,Collier JL

更新日期：2018-07-02 00:00:00

abstract：:Two-component signal transduction pathways comprising histidine protein kinases (HPKs) and their response regulators (RRs) are widely used to control bacterial responses to environmental challenges. Some bacteria have over 150 different two-component pathways, and the specificity of the phosphotransfer reactions withi...

journal_title：PLoS biology

authors： Bell CH,Porter SL,Strawson A,Stuart DI,Armitage JP

更新日期：2010-02-09 00:00:00

abstract：:Using DNA sequences 5' to open reading frames, we have constructed green fluorescent protein (GFP) fusions and generated spatial and temporal tissue expression profiles for 1,886 specific genes in the nematode Caenorhabditis elegans. This effort encompasses about 10% of all genes identified in this organism. GFP-expre...

journal_title：PLoS biology

authors： Hunt-Newbury R,Viveiros R,Johnsen R,Mah A,Anastas D,Fang L,Halfnight E,Lee D,Lin J,Lorch A,McKay S,Okada HM,Pan J,Schulz AK,Tu D,Wong K,Zhao Z,Alexeyenko A,Burglin T,Sonnhammer E,Schnabel R,Jones SJ,Marra MA,

更新日期：2007-09-01 00:00:00

abstract：:Modeling the processes of neuronal progenitor proliferation and differentiation to produce mature cortical neuron subtypes is essential for the study of human brain development and the search for potential cell therapies. We demonstrated a novel paradigm for the generation of vascularized organoids (vOrganoids) consis...

journal_title：PLoS biology

authors： Shi Y,Sun L,Wang M,Liu J,Zhong S,Li R,Li P,Guo L,Fang A,Chen R,Ge WP,Wu Q,Wang X

更新日期：2020-05-13 00:00:00

abstract：:In eukaryotes, the highly conserved U3 small nucleolar RNA (snoRNA) base-pairs to multiple sites in the pre-ribosomal RNA (pre-rRNA) to promote early cleavage and folding events. Binding of the U3 box A region to the pre-rRNA is mutually exclusive with folding of the central pseudoknot (CPK), a universally conserved r...

journal_title：PLoS biology

authors： Sardana R,Liu X,Granneman S,Zhu J,Gill M,Papoulas O,Marcotte EM,Tollervey D,Correll CC,Johnson AW

更新日期：2015-02-24 00:00:00

abstract：:The sperm's crucial function is to locate and fuse with a mature oocyte. Under laboratory conditions, Caenorhabditis elegans sperm are very efficient at navigating the hermaphrodite reproductive tract and locating oocytes. Here, we identify chemosensory and oxygen-sensing circuits that affect the sperm's navigational ...

journal_title：PLoS biology

authors： Hoang HD,Miller MA

更新日期：2017-06-29 00:00:00

abstract：:The source of symmetry breaking in vertebrate oocytes is unknown. Animal-vegetal oocyte polarity is established by the Balbiani body (Bb), a conserved structure found in all animals examined that contains an aggregate of specific mRNAs, proteins, and organelles. The Bb specifies the oocyte vegetal pole, which is key t...

journal_title：PLoS biology

authors： Elkouby YM,Jamieson-Lucy A,Mullins MC

更新日期：2016-01-07 00:00:00

abstract：:Misalignment of the daily sleep-wake and fasting-feeding cycles with the endogenous circadian timing system is an inevitable consequence of night shift work and is associated with adverse metabolic health effects. However, a detailed characterisation of the effects of night shifts on 24-h rhythms in the metabolome is ...

journal_title：PLoS biology

authors： Kervezee L,Cermakian N,Boivin DB

更新日期：2019-06-18 00:00:00

abstract：:Over the past decade, the high-dose refuge (HDR) strategy, aimed at delaying the evolution of pest resistance to Bacillus thuringiensis (Bt) toxins produced by transgenic crops, became mandatory in the United States and is being discussed for Europe. However, precopulatory dispersal and the mating rate between residen...

journal_title：PLoS biology

authors： Dalecky A,Ponsard S,Bailey RI,Pélissier C,Bourguet D

更新日期：2006-06-01 00:00:00

abstract：:Independently evolving populations may adapt to similar selection pressures via different genetic changes. The interactions between such changes, such as in a hybrid individual, can inform us about what course adaptation may follow and allow us to determine whether gene flow would be facilitated or hampered following ...

journal_title：PLoS biology

authors： Ono J,Gerstein AC,Otto SP

更新日期：2017-01-23 00:00:00

abstract：:Gene expression in eukaryotes is controlled by DNA sequences at promoter and enhancer regions, whose accessibility for binding by regulatory proteins dictates their specific patterns of activity. Here, we identify the protein Zbtb7a as a factor required for inducible changes in accessibility driven by transcription fa...

journal_title：PLoS biology

authors： Ramos Pittol JM,Oruba A,Mittler G,Saccani S,van Essen D

更新日期：2018-05-29 00:00:00

abstract：:Self-incompatibility (SI) is the flowering plant reproductive system in which self pollen tube growth is inhibited, thereby preventing self-fertilization. SI has evolved independently in several different flowering plant lineages. In all Brassicaceae species in which the molecular basis of SI has been investigated in ...

journal_title：PLoS biology

authors： Chantha SC,Herman AC,Platts AE,Vekemans X,Schoen DJ

更新日期：2013-01-01 00:00:00

abstract：:Unc-51-like autophagy activating kinase 1 (ULK1)-autophagy-related 13 (ATG13) is the most upstream autophagy initiation complex that is phosphorylated by mammalian target-of-rapamycin complex 1 (mTORC1) and AMP-activated protein kinase (AMPK) to induce autophagy in asynchronous conditions. However, their phospho-regul...

journal_title：PLoS biology

authors： Li Z,Tian X,Ji X,Wang J,Chen H,Wang D,Zhang X

更新日期：2020-06-09 00:00:00

abstract：:The ability to identify and react to novelty within the environment is fundamental to survival. Computational models emphasize the potential role of the hippocampus in novelty detection, its unique anatomical circuitry making it ideally suited to act as a comparator between past and present experience. The hippocampus...

journal_title：PLoS biology

authors： Kumaran D,Maguire EA

更新日期：2006-11-01 00:00:00

abstract：:The small GTPase Rab5 is a key regulator of clathrin-mediated endocytosis. On early endosomes, within a spatially restricted domain enriched in phosphatydilinositol-3-phosphate [PI(3)P], Rab5 coordinates a complex network of effectors that functionally cooperate in membrane tethering, fusion, and organelle motility. H...

journal_title：PLoS biology

authors： Schnatwinkel C,Christoforidis S,Lindsay MR,Uttenweiler-Joseph S,Wilm M,Parton RG,Zerial M

更新日期：2004-09-01 00:00:00

abstract：:Proprotein convertase subtilisin/kexin type 9 (PCSK9) plays a key role in regulating the levels of plasma low-density lipoprotein cholesterol (LDL-C). Here, we demonstrate that the compound PF-06446846 inhibits translation of PCSK9 by inducing the ribosome to stall around codon 34, mediated by the sequence of the nasc...

journal_title：PLoS biology

authors： Lintner NG,McClure KF,Petersen D,Londregan AT,Piotrowski DW,Wei L,Xiao J,Bolt M,Loria PM,Maguire B,Geoghegan KF,Huang A,Rolph T,Liras S,Doudna JA,Dullea RG,Cate JH

更新日期：2017-03-21 00:00:00

abstract：:Translation termination at premature termination codons (PTCs) triggers degradation of the aberrant mRNA, but the mechanism by which a termination event is defined as premature is still unclear. Here we show that the physical distance between the termination codon and the poly(A)-binding protein PABPC1 is a crucial de...

journal_title：PLoS biology

authors： Eberle AB,Stalder L,Mathys H,Orozco RZ,Mühlemann O

更新日期：2008-04-29 00:00:00

abstract：:Central mechanisms by which specific motor programs are selected to achieve meaningful behaviors are not well understood. Using electrophysiological recordings from pharyngeal nerves upon central activation of neurotransmitter-expressing cells, we show that distinct neuronal ensembles can regulate different feeding mo...

journal_title：PLoS biology

authors： Schoofs A,Hückesfeld S,Schlegel P,Miroschnikow A,Peters M,Zeymer M,Spieß R,Chiang AS,Pankratz MJ

更新日期：2014-06-24 00:00:00

abstract：:CD1d-mediated presentation of glycolipid antigens to T cells is capable of initiating powerful immune responses that can have a beneficial impact on many diseases. Molecular analyses have recently detailed the lipid antigen recognition strategies utilized by the invariant Vα24-Jα18 TCR rearrangements of iNKT cells, wh...

journal_title：PLoS biology

authors： López-Sagaseta J,Kung JE,Savage PB,Gumperz J,Adams EJ

更新日期：2012-01-01 00:00:00

abstract：:The Hedgehog signaling is a determinant pathway for tumor progression. However, while inhibition of the Hedgehog canonical pathway-Patched-Smoothened-Gli-has proved efficient in human tumors with activating mutations in this pathway, recent clinical data have failed to show any benefit in other cancers, even though So...

journal_title：PLoS biology

authors： Delloye-Bourgeois C,Gibert B,Rama N,Delcros JG,Gadot N,Scoazec JY,Krauss R,Bernet A,Mehlen P

更新日期：2013-01-01 00:00:00

abstract：:The ability of cells to accurately control gene expression levels in response to extracellular cues is limited by the inherently stochastic nature of transcriptional regulation. A change in transcription factor (TF) activity results in changes in the expression of its targets, but the way in which cell-to-cell variabi...

journal_title：PLoS biology

authors： Carey LB,van Dijk D,Sloot PM,Kaandorp JA,Segal E

更新日期：2013-01-01 00:00:00

abstract：:A developing plant organ exhibits complex spatiotemporal patterns of growth, cell division, cell size, cell shape, and organ shape. Explaining these patterns presents a challenge because of their dynamics and cross-correlations, which can make it difficult to disentangle causes from effects. To address these problems,...

journal_title：PLoS biology

authors： Fox S,Southam P,Pantin F,Kennaway R,Robinson S,Castorina G,Sánchez-Corrales YE,Sablowski R,Chan J,Grieneisen V,Marée AFM,Bangham JA,Coen E

更新日期：2018-11-01 00:00:00

abstract：:Cilia play critical roles during embryonic development and adult homeostasis. Dysfunction of cilia leads to various human genetic diseases, including many caused by defects in transition zones (TZs), the "gates" of cilia. The evolutionarily conserved TZ component centrosomal protein 290 (CEP290) is the most frequently...

journal_title：PLoS biology

authors： Wu Z,Pang N,Zhang Y,Chen H,Peng Y,Fu J,Wei Q

更新日期：2020-12-28 00:00:00

abstract：:Kabuki Syndrome patients have a spectrum of congenital disorders, including congenital heart defects, the primary determinant of mortality. Seventy percent of Kabuki Syndrome patients have mutations in the histone methyl-transferase KMT2D. However, the underlying mechanisms that drive these congenital disorders are un...

journal_title：PLoS biology

authors： Serrano MLA,Demarest BL,Tone-Pah-Hote T,Tristani-Firouzi M,Yost HJ

更新日期：2019-09-03 00:00:00