Knockdown of genes involved in axonal transport enhances the toxicity of human neuromuscular disease-linked MATR3 mutations in Drosophila.

Abstract:

:Mutations in the nuclear matrix protein Matrin 3 (MATR3) have been identified in amyotrophic lateral sclerosis and myopathy. To investigate the mechanisms underlying MATR3 mutations in neuromuscular diseases and efficiently screen for modifiers of MATR3 toxicity, we generated transgenic MATR3 flies. Our findings indicate that expression of wild-type or mutant MATR3 in motor neurons reduces climbing ability and lifespan of flies, while their expression in indirect flight muscles (IFM) results in abnormal wing positioning and muscle degeneration. In both motor neurons and IFM, mutant MATR3 expression results in more severe phenotypes than wild-type MATR3, demonstrating that the disease-linked mutations confer pathogenicity. We conducted a targeted candidate screen for modifiers of the MATR3 abnormal wing phenotype and identified multiple enhancers involved in axonal transport. Knockdown of these genes enhanced protein levels and insolubility of mutant MATR3. These results suggest that accumulation of mutant MATR3 contributes to toxicity and implicate axonal transport dysfunction in disease pathogenesis.

journal_name

FEBS Lett

journal_title

FEBS letters

authors

Zhao M,Kao CS,Arndt C,Tran DD,Cho WI,Maksimovic K,Chen XXL,Khan M,Zhu H,Qiao J,Peng K,Hong J,Xu J,Kim D,Kim JR,Lee J,van Bruggen R,Yoon WH,Park J

doi

10.1002/1873-3468.13858

subject

Has Abstract

pub_date

2020-09-01 00:00:00

pages

2800-2818

issue

17

eissn

0014-5793

issn

1873-3468

journal_volume

594

pub_type

杂志文章
  • Pseudovitamin B(12) is the corrinoid produced by Lactobacillus reuteri CRL1098 under anaerobic conditions.

    abstract::We have reported previously on the ability of Lactobacillus reuteri to produce a compound with vitamin B(12) activity. Here we report on the chemical characterisation of this corrinoid-like molecule. High performance liquid chromatography coupled to an ultraviolet diode array detector, mass spectrometry and nuclear ma...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/j.febslet.2007.09.012

    authors: Santos F,Vera JL,Lamosa P,de Valdez GF,de Vos WM,Santos H,Sesma F,Hugenholtz J

    更新日期:2007-10-16 00:00:00

  • Persistent TNF-alpha exposure impairs store operated calcium influx in CD4+ T lymphocytes.

    abstract::Persistent tumour necrosis factor alpha (TNF-alpha) exposure uncouples proximal T-cell receptor (TCR)-signalling events. Here, we demonstrate that chronic TNF-alpha exposure also attenuates signalling distal to the TCR, by specifically inhibiting Ca2+ influx evoked by thapsigargin in CD4+ T-cells. Mitogen-induced Ca2+...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/j.febslet.2005.01.051

    authors: Church LD,Goodall JE,Rider DA,Bacon PA,Young SP

    更新日期:2005-02-28 00:00:00

  • Coupling of viral membrane proteins to phosphatidylinositide signalling system.

    abstract::C6 rat glioma cells persistently infected with subacute sclerosing panencephalitis virus (C6/SSPE) were treated with measles antiserum and purified anti-measles IgG. This stimulated phosphoinositide breakdown and an increase in inositol phosphates. In uninfected C6 cells, however, only fetal calf serum (FCS), but not ...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/0014-5793(89)81330-4

    authors: Weinmann-Dorsch C,Koschel K

    更新日期:1989-04-24 00:00:00

  • Perspectives on the automatic design of regulatory systems for synthetic biology.

    abstract::Automatic design is based on computational modeling and optimization methods to provide prototype designs to targeted problems in an unsupervised manner. For biological circuits, we need to produce quantitative predictions of cell behavior for a given genotype as consequence of the different molecular interactions. Au...

    journal_title:FEBS letters

    pub_type: 杂志文章,评审

    doi:10.1016/j.febslet.2012.02.031

    authors: Rodrigo G,Carrera J,Landrain TE,Jaramillo A

    更新日期:2012-07-16 00:00:00

  • Molecular characterization of a novel human PDZ domain protein with homology to INAD from Drosophila melanogaster.

    abstract::PDZ domains are thought to act as protein-binding modules mediating the clustering of membrane and membrane-associated proteins. The INAD protein has been shown to interact via a PDZ domain with the calcium channel TRP which contributes to capacitative calcium entry into Drosophila photoreceptor cells. We have cloned ...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/s0014-5793(97)00877-6

    authors: Philipp S,Flockerzi V

    更新日期:1997-08-18 00:00:00

  • Identification of an uncoupling mutation affecting the b subunit of F1F0 ATP synthase in Escherichia coli.

    abstract::A specific b subunit arginine, b(Arg-36) in Escherichia coli, displays evolutionary conservation among bacterial F1F0 ATP synthases. Site-directed mutagenesis was used to generate a collection of mutations affecting b(Arg-36). The phenotype differed depending upon the substitution, and the b(Arg-36-Glu) and b(Arg-36-I...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/s0014-5793(98)00597-3

    authors: Caviston TL,Ketchum CJ,Sorgen PL,Nakamoto RK,Cain BD

    更新日期:1998-06-12 00:00:00

  • MRI-guided gene therapy.

    abstract::MRI has the ability to generate high-contrast and high-resolution images, to obtain multiple diagnostic evaluations of organ function and morphology, and to provide multiple image planes with no risk of ionizing radiation. Recent efforts have focused on using MR technology to monitor gene delivery, to enhance gene tra...

    journal_title:FEBS letters

    pub_type: 杂志文章,评审

    doi:10.1016/j.febslet.2006.04.027

    authors: Yang X,Atalar E

    更新日期:2006-05-22 00:00:00

  • The secondary structure of bacteriorhodopsin in organic solution. A Fourier transform infrared study.

    abstract::Fourier transform infrared spectroscopy is used to estimate the secondary structure of bacteriorhodopsin dissolved in chloroform-methanol (1:1 v/v), 0.1 M LiClO4. Curve-fitting of the deconvolved spectra in the amide I region shows that the total content of alpha-helices, reverse turns and beta-sheets are similar to t...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/0014-5793(93)81331-s

    authors: Torres J,Padrós E

    更新日期:1993-02-22 00:00:00

  • Precise mapping of the tms1 binding site on p53.

    abstract::Originally identified as multicopy suppressor of a lethal growth arrest caused by expression of a tumour mutant cDNA of p53 in fission yeast the tms1 gene product was found to form stable complexes with p53 in yeast. By using purified recombinant proteins multimeric complexes of tms1 and p53 could be demonstrated and ...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/0014-5793(95)01329-6

    authors: Wagner P,Fuchs A,Prowald A,Montenarh M,Nastainczyk W

    更新日期:1995-12-18 00:00:00

  • Over expression of PPP2R2C inhibits human glioma cells growth through the suppression of mTOR pathway.

    abstract::PPP2R2C encodes a gamma isoform of the subunit B55 subfamily, which is a regulatory subunit of Protein phosphatase type 2A (PP2A). Our study shows that PPP2R2C is downregulated in glioma cells and human brain cancer patient samples. Overexpression of PPP2R2C inhibited cancer cell proliferation both in vitro and in viv...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/j.febslet.2013.09.029

    authors: Fan YL,Chen L,Wang J,Yao Q,Wan JQ

    更新日期:2013-12-11 00:00:00

  • Light-dependent delta mu Na-generation and utilization in the marine cyanobacterium Oscillatoria brevis.

    abstract::Light-dependent Na+ and H+ transports, membrane potential (delta psi) and motility have been studied in the cells of the marine cyanobacterium Oscillatoria brevis. In the presence of a protonophorous uncoupler, carbonyl cyanide-m-chlorophenylhydrazone, the intracellular Na+ level is shown to increase in the dark and d...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/0014-5793(90)81268-s

    authors: Brown II,Fadeyev SI,Kirik II,Severina II,Skulachev VP

    更新日期:1990-09-17 00:00:00

  • Functional argument for the existence of an avian nitric oxide synthase in muscle mitochondria: effect of cold acclimation.

    abstract::We report the first evidence of a mitochondrial NO synthase (mtNOS) in bird skeletal muscle. In vitro, mtNOS activity stimulated by L-arginine reduced intermyofibrillar mitochondrial oxygen uptake and ATP synthesis rates, stimulated endogenous H(2)O(2) generation, but had no effect on oxidative phosphorylation efficie...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/j.febslet.2010.11.032

    authors: Rey B,Roussel D,Teulier L,Eyenga P,Degletagne C,Belouze M,Duchamp C

    更新日期:2011-01-03 00:00:00

  • A new class of inhibitors of human leucocyte elastase.

    abstract::Studies of the inhibition of elastases at a molecular level have resulted in the identification of protected dipeptides which are reversible and highly specific inhibitors of human leucocyte elastase (HLE). These have been further developed by increasing their hydrophilicity and potency to give a new family of elastas...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/0014-5793(85)80776-6

    authors: Hassall CH,Johnson WH,Kennedy AJ,Roberts NA

    更新日期:1985-04-22 00:00:00

  • A FoxO1-dependent, but NRF2-independent induction of heme oxygenase-1 during muscle atrophy.

    abstract::Skeletal muscle plays key roles in metabolic homeostasis. Loss of muscle mass, called muscle atrophy exacerbates disease-associated metabolic perturbations. In this study, we characterized the molecular functions and mechanisms underlying regulation of skeletal muscle atrophy induced by denervation. Denervation signif...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/j.febslet.2013.11.009

    authors: Kang J,Jeong MG,Oh S,Jang EJ,Kim HK,Hwang ES

    更新日期:2014-01-03 00:00:00

  • The neurotoxin histrionicotoxin interacts with the putative ion channel of the nicotinic acetylcholine receptors in the central nervous system.

    abstract::Perhydrohistrionicotoxin at micromolar concentrations blocked the nicotine-evoked transmitter release from perfused striatal (dopaminergic) and hippocampal (cholinergic) nerve terminals. Perhydrohistrionicotoxin failed to compete with [3H]nicotine for its high-affinity binding site in rat brain, suggesting that the ac...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/0014-5793(87)81363-7

    authors: Rapier C,Wonnacott S,Lunt GG,Albuquerque EX

    更新日期:1987-02-23 00:00:00

  • In vitro endosome-lysosome transfer of dephosphorylated EGF receptor and Shc in rat liver.

    abstract::We have studied the endosome-lysosome transfer of internalized epidermal growth factor receptor (EGFR) complexes in a cell-free system from rat liver. Analytical subfractionation of a postmitochondrial supernatant fraction showed that a pulse of internalized [(125)I]EGF was largely associated with a light endosomal fr...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/s0014-5793(99)01413-1

    authors: Authier F,Chauvet G

    更新日期:1999-11-12 00:00:00

  • Sequence analysis of the promoter region of the rat vasopressin gene.

    abstract::The vasopressin gene is highly transcribed in magnocellular neurons of the supraoptic (SON) and paraventricular nucleus (PVN) in the rat hypothalamus. In order to identify cis-acting elements involved in the expression of the vasopressin gene, approximately 1 kb upstream of the transcription start site has been sequen...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/0014-5793(90)80130-b

    authors: Mohr E,Richter D

    更新日期:1990-01-29 00:00:00

  • Penicillin-binding proteins of beta-lactam-resistant strains of Staphylococcus aureus. Effect of growth conditions.

    abstract::Methicillin-resistant clinical isolates of Staphylococcus aureus are intrinsically resistant to beta-lactam antibiotics in that the resistance mechanism is unrelated to the possession of beta-lactamases. We have demonstrated that a new, high-molecular-mass penicillin-binding protein (PBP) is present in these strains w...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/0014-5793(85)80036-3

    authors: Reynolds PE,Brown DF

    更新日期:1985-11-11 00:00:00

  • Calcium signaling mediates the response to cadmium toxicity in Saccharomyces cerevisiae cells.

    abstract::The involvement of Ca(2+) in the response to high Mn(2+), Co(2+), Ni(2+), Cu(2+), Zn(2+), Cd(2+), and Hg(2+) was investigated in Saccharomyces cerevisiae. The yeast cells responded through a sharp increase in cytosolic Ca(2+) when exposed to Cd(2+), and to a lesser extent to Cu(2+), but not to Mn(2+), Co(2+), Ni(2+), ...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/j.febslet.2014.07.001

    authors: Ruta LL,Popa VC,Nicolau I,Danet AF,Iordache V,Neagoe AD,Farcasanu IC

    更新日期:2014-08-25 00:00:00

  • Electrostatic influence of active-site waters on the nucleophilic aromatic substitution catalyzed by 4-chlorobenzoyl-CoA dehalogenase.

    abstract::The 4-chlorobenzoyl-CoA dehalogenase catalyzes the hydrolytic dechlorination of 4-chlorobenzoyl-CoA via a two-step mechanism, namely nucleophilic aromatic substitution and ester hydrolysis. The mutation of an active-site Histidine residue has been shown to reduce the catalytic activity in both the substitution and sub...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/j.febslet.2005.06.056

    authors: Xu D,Guo H

    更新日期:2005-08-15 00:00:00

  • Studies of a putative ice-binding motif in winter flounder skin-type anti-freeze polypeptide.

    abstract::Winter flounder contains two distinct anti-freeze protein isoforms, which are the liver-type extracellular anti-freeze proteins and the skin-type intracellular anti-freeze protein. The skin-type anti-freeze proteins exhibit lower anti-freeze activities than the liver-type isoforms and this might be due to their lackin...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/s0014-5793(99)00752-8

    authors: Lin Q,Ewart KV,Yang DS,Hew CL

    更新日期:1999-06-25 00:00:00

  • The mechanisms and regulation of procathepsin L secretion from osteoclasts in bone resorption.

    abstract::The secretion mechanisms of cathepsin L from osteoclasts in the process of bone resorption were investigated. The increases in bone pit numbers formed take place by PTH addition in parallel with the increases of cathepsin L and/or L-like proteinase activities in the culture medium of bone cells, and these were suppres...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/0014-5793(94)80522-9

    authors: Tagami K,Kakegawa H,Kamioka H,Sumitani K,Kawata T,Lenarcic B,Turk V,Katunuma N

    更新日期:1994-04-11 00:00:00

  • Lipopolysaccharide signaling induces serum amyloid A (SAA) synthesis in human hepatocytes in vitro.

    abstract::To investigate the role of lipopolysaccharide (LPS) in hepatocyte activation, we examined the expression of Toll-like receptor 4 (TLR4), the putative receptor for LPS in human hepatocytes. TLR4 mRNA and protein expression was confirmed in human hepatocytes. Stimulation of human hepatocytes with LPS results in rapid de...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/j.febslet.2004.05.072

    authors: Migita K,Abiru S,Nakamura M,Komori A,Yoshida Y,Yokoyama T,Daikoku M,Ueki T,Takii Y,Yano K,Yastuhashi H,Eguchi K,Ishibashi H

    更新日期:2004-07-02 00:00:00

  • Molecular cloning of a novel protein-tyrosine phosphatase SH-PTP3 with sequence similarity to the src-homology region 2.

    abstract::Protein-tyrosine phosphorylation and dephosphorylation are directly associated with cellular growth, signal transduction, and neoplastic transformation. Here we report the isolation of a complementary DNA (cDNA) clone encoding a novel protein-tyrosine phosphatase (PTP) from a human T cell PEER cDNA library. The predic...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/0014-5793(92)81500-l

    authors: Adachi M,Sekiya M,Miyachi T,Matsuno K,Hinoda Y,Imai K,Yachi A

    更新日期:1992-12-21 00:00:00

  • Cloning and sequence of full-length cDNAs encoding the human neuronal nicotinic acetylcholine receptor (nAChR) subunits beta3 and beta4 and expression of seven nAChR subunits in the human neuroblastoma cell line SH-SY5Y and/or IMR-32.

    abstract::Using PCR-based techniques, we have isolated and sequenced the full-length cDNAs that encode the human neuronal nAChR subunits alpha3,4,5,7 and beta2,3,4 in the neuroblastoma cell lines SH-SY5Y and/or IMR-32. The predicted nAChR beta3- and beta4-subunit proteins contain 458 and 498 amino acids, respectively. Except fo...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/s0014-5793(96)01383-x

    authors: Groot Kormelink PJ,Luyten WH

    更新日期:1997-01-06 00:00:00

  • Glucosyl epi-cyclophellitol allows mechanism-based inactivation and structural analysis of human pancreatic α-amylase.

    abstract::As part of a search for selective, mechanism-based covalent inhibitors of human pancreatic α-amylase we describe the chemoenzymatic synthesis of the disaccharide analog α-glucosyl epi-cyclophellitol, demonstrate its stoichiometric reaction with human pancreatic α-amylase and evaluate the time dependence of its inhibit...

    journal_title:FEBS letters

    pub_type: 信件

    doi:10.1002/1873-3468.12143

    authors: Caner S,Zhang X,Jiang J,Chen HM,Nguyen NT,Overkleeft H,Brayer GD,Withers SG

    更新日期:2016-04-01 00:00:00

  • The sodium ion pumping oxaloacetate decarboxylase of Klebsiella pneumoniae. Metal ion content, inhibitors and proteolytic degradation studies.

    abstract::Oxaloacetate decarboxylase of Klebsiella pneumoniae was shown to contain between 0.6 and 1.0 mol zinc per mol enzyme in different preparations. The decarboxylase activity was completely abolished after 15 min incubation with 1 mM Hg(NO3)2 in phosphate buffer, while the activity decreased only 20% if the incubation was...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/0014-5793(92)80165-d

    authors: Dimroth P,Thomer A

    更新日期:1992-03-23 00:00:00

  • Formation of 22 and 24 carbon 6-desaturated fatty acids from exogenous deuterated arachidonic acid is activated in THP-1 cells at high substrate concentrations.

    abstract::Deuterated arachidonic acid (AA, [2H8]20:4 n-6) 1-25 microM, is converted to other fatty acids, as evaluated by gas chromatography-mass spectrometry, in THP-1 cells. The major products, in the 1 to 10 microM range, are 22:4 (elongated) and 20:3 (reduced in 5). At 25 microM, 24:4, 24:5 and 22:5 accumulate, with [2H8]/[...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/0014-5793(94)80554-7

    authors: Caruso D,Risé P,Galella G,Regazzoni C,Toia A,Galli G,Galli C

    更新日期:1994-05-02 00:00:00

  • Molecular mechanism of inhibition of mammalian protein synthesis by some four-chain agglutinins. Proposal of an extended classification of plant ribosome-inactivating proteins (rRNA N-glycosidases).

    abstract::The four chain agglutinins from Abrus precatorius, Viscum album and Ricinus communis promote depurination of the 28 S rRNA from rabbit reticulocyte ribosomes characteristic of the common ribosome-inactivating proteins (RIPs). These agglutinins inhibited mammalian protein synthesis at nanomolar concentrations but they ...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/0014-5793(93)80193-x

    authors: Citores L,Ferreras JM,Iglesias R,Carbajales ML,Arias FJ,Jiménez P,Rojo MA,Girbés T

    更新日期:1993-08-23 00:00:00

  • Involvement of electrostatic phenomena in phospholamban-induced stimulation of Ca uptake into cardiac sarcoplasmic reticulum.

    abstract::The activity of the Ca-pumping ATPase of cardiac sarcoplasmic reticulum (SR) is controlled by the phosphorylation of the intrinsic regulatory protein phospholamban (PLB), which affects both the apparent Km(Ca) and the Vmax of the transport process. We have investigated the correlation between phosphorylation of PLB an...

    journal_title:FEBS letters

    pub_type: 杂志文章

    doi:10.1016/0014-5793(89)81201-3

    authors: Chiesi M,Schwaller R

    更新日期:1989-02-13 00:00:00