Aggregation of ALS-linked FUS mutant sequesters RNA binding proteins and impairs RNA granules formation.

Abstract:

:Protein aggregate/inclusion is one of hallmarks for neurodegenerative disorders including amyotrophic lateral sclerosis (ALS). FUS/TLS, one of causative genes for familial ALS, encodes a multifunctional DNA/RNA binding protein predominantly localized in the nucleus. C-terminal mutations in FUS/TLS cause the retention and the inclusion of FUS/TLS mutants in the cytoplasm. In the present study, we examined the effects of ALS-linked FUS mutants on ALS-associated RNA binding proteins and RNA granules. FUS C-terminal mutants were diffusely mislocalized in the cytoplasm as small granules in transiently transfected SH-SY5Y cells, whereas large aggregates were spontaneously formed in ∼10% of those cells. hnRNP A1, hnRNP A2, and SMN1 as well as FUS wild type were assembled into stress granules under stress conditions, and these were also recruited to FUS mutant-derived spontaneous aggregates in the cytoplasm. These aggregates stalled poly(A) mRNAs and sequestered SMN1 in the detergent insoluble fraction, which also reduced the number of nuclear oligo(dT)-positive foci (speckles) in FISH (fluorescence in situ hybridization) assay. In addition, the number of P-bodies was decreased in cells harboring cytoplasmic granules of FUS P525L. These findings raise the possibility that ALS-linked C-terminal FUS mutants could sequester a variety of RNA binding proteins and mRNAs in the cytoplasmic aggregates, which could disrupt various aspects of RNA equilibrium and biogenesis.

authors

Takanashi K,Yamaguchi A

doi

10.1016/j.bbrc.2014.08.115

subject

Has Abstract

pub_date

2014-09-26 00:00:00

pages

600-7

issue

3

eissn

0006-291X

issn

1090-2104

pii

S0006-291X(14)01548-4

journal_volume

452

pub_type

杂志文章
  • Suppressive effect of transforming growth factor-beta on the phosphorylation of endogenous substrates by conventional and novel protein kinase C in primary cultured mouse epidermal cells.

    abstract::The effect of transforming growth factor-beta (TGF-beta) on the endogenous protein phosphorylation caused by phorbol 12-myristate 13-acetate (PMA), a potent activator of protein kinase C (PKC), was examined in primary cultured mouse epidermal cells. PMA markedly stimulates phosphorylation of endogenous proteins, i.e. ...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1006/bbrc.1993.1635

    authors: Nishikawa K,Yamamoto S,Nagumo H,Otsuka C,Kato R

    更新日期:1993-05-28 00:00:00

  • Isolation and characterization of a carcinoma-associated antigen.

    abstract::GA733 is a murine IgG2a monoclonal antibody (MAb) against human gastric carcinoma and is highly tumoricidal in nude mice. The GA733 antigen is a cell surface protein with two subunits of 30,000 and 40,000 daltons. The antigen isolated by immunoaffinity chromatography consists mainly of the 30,000-dalton subunit which ...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/0006-291x(86)90976-9

    authors: Ross AH,Herlyn D,Iliopoulos D,Koprowski H

    更新日期:1986-02-26 00:00:00

  • AIMP1 regulates TCR signaling and induces differentiation of regulatory T cells by interfering with lipid raft association.

    abstract::In addition to a role in translation, AIMP1 is secreted to affect various immune cells, such as macrophages, dendritic cells, B cells, and natural killer cells. However, the direct effects of AIMP1 on T cells have not yet been reported. In this study, we investigated whether AIMP1 could modulate T cell responses direc...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/j.bbrc.2019.05.040

    authors: Kim MS,Lee A,Cho D,Kim TS

    更新日期:2019-06-30 00:00:00

  • Deltoid human muscle mtDNA is extensively rearranged in old age subjects.

    abstract::Extra long PCR analysis of mitochondrial DNA (mtDNA) isolated from skeletal muscle of humans of different ages revealed three phenomena: (i) the amount of normal length mtDNA (16.5 kb) was progressively reduced with age, such that the cells of old age individuals (about 90 years) contained little, or undetectable amou...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1006/bbrc.1997.6251

    authors: Kovalenko SA,Kopsidas G,Kelso JM,Linnane AW

    更新日期:1997-03-06 00:00:00

  • Tissue distribution and characterization of prorenin-converting enzyme in mouse.

    abstract::Renin is produced from an inactive precursor, prorenin, through endoproteolytic cleavage at paired basic amino-acid residues. Using (35S)methionine-labeled prorenin, that was synthesized with Xenopus oocyte expression system, as a substrate, we have determined the tissue distribution and the nature of prorenin-convert...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/s0006-291x(89)80057-9

    authors: Nakayama K,Kim WS,Hatsuzawa K,Hashiba K,Murakami K

    更新日期:1989-01-31 00:00:00

  • COMP-Ang1 ameliorates leukocyte adhesion and reinforces endothelial tight junctions during endotoxemia.

    abstract::Endotoxemia is characterized by multiple dysfunctions of the micro-vascular endothelium. Of these, vascular leakage is an initial event that aggravates vascular dysfunction can lead to systemic vascular collapse and organ failure. Thus, prevention of vascular leakage may ameliorate endotoxin-induced dysfunctions of bl...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/j.bbrc.2009.02.096

    authors: Hwang JA,Lee EH,Lee SD,Park JB,Jeon BH,Cho CH

    更新日期:2009-04-17 00:00:00

  • Interleukin-1beta-induced apoptosis through adenylyl cyclase and ERK1/2 inhibition in primary cultured thyroid cells.

    abstract::The programmed cell death plays a crucial role in the regulation of numerous physiological and pathological phenomena. In this study, we show that interleukin-1 beta (IL-1beta) induces an early production of endogenous ceramides via N-sphingomyelinase (N-Smase) as well as an inhibition of adenylyl cyclase activity in ...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/j.bbrc.2005.10.213

    authors: El Btaouri H,Rath G,Morjani H,Schneider C,Petitfrere E,Antonicelli F,Martiny L

    更新日期:2006-01-13 00:00:00

  • Intraperitoneal AAV9-shRNA inhibits target expression in neonatal skeletal and cardiac muscles.

    abstract::Systemic injections of AAV vectors generally transduce to the liver more effectively than to cardiac and skeletal muscles. The short hairpin RNA (shRNA)-expressing AAV9 (shRNA-AAV9) can also reduce target gene expression in the liver, but not enough in cardiac or skeletal muscles. Higher doses of shRNA-AAV9 required f...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/j.bbrc.2011.01.009

    authors: Mayra A,Tomimitsu H,Kubodera T,Kobayashi M,Piao W,Sunaga F,Hirai Y,Shimada T,Mizusawa H,Yokota T

    更新日期:2011-02-11 00:00:00

  • Trans-activation of herpes simplex virus type 1 immediate early genes is specifically inhibited by human recombinant interferons.

    abstract::The effect of human recombinant interferons (IFNs) alpha and gamma on the transcription of Herpes Simplex virus type-1 (HSV-1) immediate early (IE) genes has been studied. Human amniotic cells (WISH) were transfected with a chimaeric plasmid containing the regulatory region of the HSV-1 IE genes controlling the expres...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/0006-291x(89)90011-9

    authors: De Stasio PR,Taylor MW

    更新日期:1989-03-15 00:00:00

  • Epoxyeicosatrienoic acids protect pancreatic beta cells against pro-inflammatory cytokine toxicity.

    abstract::Pro-inflammatory cytokines contribute to pancreatic beta cell death in the pathogenesis of type 1 diabetes mellitus (DM). Cytochrome P450-derived epoxyeicosatrienoic acids (EETs), produced by selective epoxidation of arachidonic acid, display anti-inflammatory activity in numerous disease models, in part through inhib...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/j.bbrc.2019.09.124

    authors: Grimes D,Watson D

    更新日期:2019-12-03 00:00:00

  • GPR35 is a functional receptor in rat dorsal root ganglion neurons.

    abstract::GPR35, previously an orphan G-protein coupled receptor, is a receptor for kynurenic acid. Here we examine the distribution of GPR35 in the rat dorsal root ganglion (DRG) and the effects of its selective activation. GPR35 was expressed predominantly by small- to medium-diameter neurons of the DRG. Many of these same ne...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/j.bbrc.2007.10.197

    authors: Ohshiro H,Tonai-Kachi H,Ichikawa K

    更新日期:2008-01-11 00:00:00

  • Characterization of casein kinase II in human colonic carcinomas after heterotransplantation into nude mice.

    abstract::Casein kinase II (CKII) activity in colorectal tumours was compared before and after heterotransplantation onto nude mice. The test revealed that the enzyme activity was about two-fold enhanced in the tumours isolated from the nude mice when compared to the respective primary tumours from which they were derived. Immu...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/0006-291x(89)92184-0

    authors: Seitz G,Münstermann U,Schneider HR,Issinger OG

    更新日期:1989-08-30 00:00:00

  • Human VAPA and the yeast VAP Scs2p with an altered proline distribution can phenocopy amyotrophic lateral sclerosis-associated VAPB(P56S).

    abstract::A human isoform of the vesicle-associated membrane protein-associated proteins (VAPs), VAPB, causes amyotrophic lateral sclerosis eight due to the missense mutation of Pro-56, whereas human VAPA and the yeast VAP Scs2p proteins are not significantly affected by similar mutations. We have found that VAPA and Scs2p have...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/j.bbrc.2010.12.011

    authors: Nakamichi S,Yamanaka K,Suzuki M,Watanabe T,Kagiwada S

    更新日期:2011-01-14 00:00:00

  • Melanoma cell lines express VEGF receptor KDR and respond to exogenously added VEGF.

    abstract::Tumour-secreted vascular endothelial growth factor (VEGF) exerts a number of effects which are important in tumour pathology, including stimulation of angiogenesis and permeabilisation of tumour-associated vasculature. In this study we have examined the possibility that VEGF may also play an autocrine role in tumour g...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1006/bbrc.1995.2832

    authors: Liu B,Earl HM,Baban D,Shoaibi M,Fabra A,Kerr DJ,Seymour LW

    更新日期:1995-12-26 00:00:00

  • Antiproliferative effect of 1,25-dihydroxyvitamin D3 and its analogs on human colon adenocarcinoma cells (CaCo-2): influence of extracellular calcium.

    abstract::Depending on culture in either "low Ca++" (0.25 mM) or "normal Ca++" (1.8 mM) medium, human colon adenocarcinoma-derived CaCo-2 cells exhibit differential sensitivity to the antiproliferative action of 1,25-dihydroxyvitamin D3 (1,25(OH)2D3) and of two side-chain modified analogs, 1,25S,26-trihydroxy-delta 22-vitamin D...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/0006-291x(91)91333-8

    authors: Cross HS,Huber C,Peterlik M

    更新日期:1991-08-30 00:00:00

  • Semicarbazide-sensitive amine oxidase in transgenic mice with diabetes.

    abstract::Semicarbazide-sensitive amine oxidase (SSAO) activity in plasma is increased in diabetes, and in particular, in diabetic patients with vascular complications. It has been speculated that SSAO is involved in the development of such complications due to the production of cytotoxic compounds. In this work, we have induce...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/j.bbrc.2004.10.140

    authors: Gokturk C,Nordquist J,Sugimoto H,Forsberg-Nilsson K,Nilsson J,Oreland L

    更新日期:2004-12-17 00:00:00

  • PIAS3 enhances the transcriptional activity of HIF-1α by increasing its protein stability.

    abstract::The transcription factor hypoxia-inducible factor-1 (HIF-1) functions as a master regulator of hypoxic response by inducing the transcription of various genes responsible for cellular adaptation to hypoxia. In this study, we investigated the effects of protein inhibitor of activated STAT3 (PIAS3), a small ubiquitin-re...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/j.bbrc.2015.12.047

    authors: Nakagawa K,Kohara T,Uehata Y,Miyakawa Y,Sato-Ueshima M,Okubo N,Asaka M,Takeda H,Kobayashi M

    更新日期:2016-01-15 00:00:00

  • Insulin resistance increases PAI-1 in the heart.

    abstract::To determine whether insulin resistance increases expression of plasminogen activator inhibitor type-1 (PAI-1) in the heart, studies were performed in 22 mice with and 38 without myocardial infarction. Insulin resistance in transgenic animals genetically rendered insulin resistant was confirmed with the use of intrape...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/j.bbrc.2006.05.078

    authors: Sobel BE,Schneider DJ,Lee YH,Pratley RE

    更新日期:2006-07-21 00:00:00

  • Physical association of Ras and cathepsins B and L in the conditioned medium of v-Ha-ras-transformed NIH3T3 cells.

    abstract::Immunoblot analysis of the conditioned medium of v-Ha-ras-transformed NIH3T3 cells showed that Ras protein is secreted in the culture fluid. Because cathepsins L and B whose activities are inhibited by Ras are also secreted, possible association between Ras and cathepsins was investigated by immunoprecipitation and fo...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1006/bbrc.1995.2696

    authors: Hiwasa T,Kominami E

    更新日期:1995-11-22 00:00:00

  • The mitogenic principle of Escherichia coli lipoprotein: B-lymphocyte mitogenicity of the synthetic analogue palmitoyl-tetrapeptide (Pam-Ser-Ser-Asn-Ala).

    abstract::N-Palmitoyl-(S)-seryl-(S)-seryl-(S)-asparaginyl-(S)-alanine (Palmitoyl-tetrapeptide) is an analogue of the N-terminal part of the lipoprotein from the outer membrane of Escherichia coli. It was prepared by chemical synthesis and tested for biological activity in in vitro lymphocyte culture systems. In spleen cells of ...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/0006-291x(84)90687-9

    authors: Bessler WG,Cox M,Wiesmüller KH,Jung G

    更新日期:1984-05-31 00:00:00

  • Remodeling of ion channel expression may contribute to electrophysiological consequences caused by methamphetamine in vitro and in vivo.

    abstract::Methamphetamine (MA) is a psychostimulant. MA may induce numerous cardiotoxic effects, leading to cardiac arrhythmias, heart failure, eventually leading to sudden cardiac death. The deleterious effects of methamphetamine work in tandem to disrupt the coordinated electrical activity of the heart and have been associate...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/j.bbrc.2013.11.114

    authors: Qu YH,Leung KP,Qiao DF,Li DR,Liu C,Yue X,Wang HJ

    更新日期:2014-01-10 00:00:00

  • Recognition of the SARS-CoV-2 receptor binding domain by neutralizing antibodies.

    abstract::Immediately from the outset of the COVID-19 pandemic, researchers from diverse biomedical and biological disciplines have united to study the novel pandemic virus, SARS-CoV-2. The antibody response to SARS-CoV-2 has been a major focus of COVID-19 research due to its clinical relevance and importance in vaccine and the...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/j.bbrc.2020.10.012

    authors: Yuan M,Liu H,Wu NC,Wilson IA

    更新日期:2020-10-10 00:00:00

  • Exercise maintains blood-brain barrier integrity during early stages of brain metastasis formation.

    abstract::Tumor cell extravasation into the brain requires passage through the blood-brain barrier, which is a highly protected microvascular environment fortified with tight junction (TJ) proteins. TJ integrity can be regulated under physiological and pathophysiological conditions. There is evidence that exercise can modulate ...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/j.bbrc.2015.04.153

    authors: Wolff G,Davidson SJ,Wrobel JK,Toborek M

    更新日期:2015-08-07 00:00:00

  • TT virus is distributed in various leukocyte subpopulations at distinct levels, with the highest viral load in granulocytes.

    abstract::When TT virus (TTV) DNA was quantitated in whole blood and plasma aliquots from 27 viremic individuals by real-time detection PCR that can detect essentially all TTV genotypes, the TTV load was 6.9 +/- 3.5 (mean +/- standard deviation)-fold higher in the whole blood than in the plasma samples [P < 0.002 (paired t test...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1006/bbrc.2001.6183

    authors: Takahashi M,Asabe S,Gotanda Y,Kishimoto J,Tsuda F,Okamoto H

    更新日期:2002-01-11 00:00:00

  • Effect of aphidicolin on the elongation step of adenovirus DNA replication in vitro.

    abstract::Adenovirus DNA synthesis carried out in vitro was inhibited by the aphidicolin. However, 30% of the DNA synthesis was resistant to aphidicolin even at a concentration of 200 micrograms/ml. When the distribution patterns of the radioactivity of the products synthesized in the presence of 50 micrograms/ml of the drug wa...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/0006-291x(83)90435-7

    authors: Ariga H

    更新日期:1983-05-31 00:00:00

  • An Arabidopsis thaliana cDNA complements the N-acetylglucosaminyltransferase I deficiency of CHO Lec1 cells.

    abstract::N-Acetylglucosaminyltransferase I (GlcNAcT-I, EC 2.4.1.101) is the enzyme which initiates the formation of complex N-linked glycans in eukaryotes by transforming GlcNAc to the oligo-mannosyl acceptor Man(5)GlcNAc(2)-Asn. The enzymatic activity and the structure that is synthesised by this enzyme are found in animals a...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1006/bbrc.1999.1117

    authors: Bakker H,Lommen A,Jordi W,Stiekema W,Bosch D

    更新日期:1999-08-11 00:00:00

  • Inverse expression of two laminin binding proteins, 67LR and galectin-3, correlates with the invasive phenotype of trophoblastic tissue.

    abstract::Tumor invasion of host tissues and trophoblastic penetration of the endometrium share common biological features. Both processes involve the invasion of basement membranes, an event that is initiated by adhesion of cancer or trophoblast cells to basement membrane components and particularly to laminin. Adhesion to thi...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1006/bbrc.1994.1713

    authors: van den Brûle FA,Price J,Sobel ME,Lambotte R,Castronovo V

    更新日期:1994-05-30 00:00:00

  • Arabidopsis chloroplast chaperonin 10 is a calmodulin-binding protein.

    abstract::Calcium regulates diverse cellular activities in plants through the action of calmodulin (CaM). By using (35)S-labeled CaM to screen an Arabidopsis seedling cDNA expression library, a cDNA designated as AtCh-CPN10 (Arabidopsis thaliana chloroplast chaperonin 10) was cloned. Chloroplast CPN10, a nuclear-encoded protein...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1006/bbrc.2000.3335

    authors: Yang T,Poovaiah BW

    更新日期:2000-08-28 00:00:00

  • Context-dependent role of ATG4B as target for autophagy inhibition in prostate cancer therapy.

    abstract::ATG4B belongs to the autophagin family of cysteine proteases required for autophagy, an emerging target of cancer therapy. Developing pharmacological ATG4B inhibitors is a very active area of research. However, detailed studies on the role of ATG4B during anticancer therapy are lacking. By analyzing PC-3 and C4-2 pros...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/j.bbrc.2013.10.117

    authors: Tran E,Chow A,Goda T,Wong A,Blakely K,Rocha M,Taeb S,Hoang VC,Liu SK,Emmenegger U

    更新日期:2013-11-29 00:00:00

  • Glycerol-3-phosphate dehydrogenase mRNA content in cultured 3T3-L1 adipocytes: regulation by dibutyryl cyclic AMP.

    abstract::Incubation of differentiated 3T3-L1 adipocytes with 0.5 mM dibutyryl cAMP plus 0.5 mM theophylline for 2 hours results in an 85% decrease in glycerol-3-phosphate dehydrogenase (G3PD) mRNA content. Incubation of the adipocytes with insulin (1 microgram/ml) up to 24 hrs yielded no significant change in the G3PD mRNA con...

    journal_title:Biochemical and biophysical research communications

    pub_type: 杂志文章

    doi:10.1016/0006-291x(85)90217-7

    authors: Bhandari B,Miller RE

    更新日期:1985-09-30 00:00:00