Post-transcriptional suppression of pathogenic prion protein expression in Drosophila neurons.


:A wealth of evidence supports the view that conformational change of the prion protein, PrPC, into a pathogenic isoform, PrPSc, is the hallmark of sporadic, infectious, and inherited forms of prion disease. Although the central role played by PrPSc in the pathogenesis of prion disease is appreciated, the cellular mechanisms that recognize PrPSc and modulate its production, clearance, and neural toxicity have not been elucidated. To address these questions, we used a tissue-specific expression system to express wild-type and disease-associated PrP molecules heterologously in Drosophila melanogaster. Our results indicate that Drosophila brain possesses a specific and saturable mechanism that suppresses the accumulation of PG14, a disease-associated insertional PrP mutant. We also found that wild-type PrP molecules are maintained in a detergent-soluble conformation throughout life in Drosophila brain neurons, whereas they become detergent-insoluble in retinal cells as flies age. PG14 protein expression in Drosophila eye did not cause retinal pathology. Our work reveals the presence of mechanisms in neurons that specifically counterbalance the production of misfolded PrP conformations, and provides an opportunity to study these processes in a model organism amenable to genetic analysis.


J Neurochem


Deleault NR,Dolph PJ,Feany MB,Cook ME,Nishina K,Harris DA,Supattapone S





Has Abstract


2003-06-01 00:00:00














  • Involvement of TG-interacting factor in microglial activation during experimental traumatic brain injury.

    abstract::Traumatic brain injury (TBI) is a complex injury involving several physiological alterations, potentially leading to neurological impairment. Previous mouse studies using high-density oligonucleotide array analysis have confirmed the upregulation of transforming growth-interacting factor (TGIF) mRNA in TBI. TGIF is a ...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Chio CC,Chang CP,Lin MT,Su FC,Yang CZ,Tseng HY,Liu ZM,Huang HS

    更新日期:2014-12-01 00:00:00

  • FUS regulates autophagy by mediating the transcription of genes critical to the autophagosome formation.

    abstract::Fused in sarcoma (FUS) is a ubiquitously expressed RNA/DNA-binding protein that plays different roles in the cell. FUS pathology has been reported in neurodegenerative diseases amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Mutations in FUS have also been linked to a subset of familial ALS. FUS...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Arenas A,Kuang L,Zhang J,Kingren MS,Zhu H

    更新日期:2020-12-22 00:00:00

  • The calcium-activated protease calpain regulates netrin-1 receptor deleted in colorectal cancer-induced axon outgrowth in cortical neurons.

    abstract::During development, neurons extend axons toward their appropriate synaptic targets to establish functional neuronal connections. The growth cone, a highly motile structure at the tip of the axon, is capable of recognizing extracellular guidance cues and translating them into directed axon outgrowth through modulation ...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Duquette PM,Lamarche-Vane N

    更新日期:2020-02-01 00:00:00

  • Microelectrode array studies of basal and potassium-evoked release of L-glutamate in the anesthetized rat brain.

    abstract::L-glutamate (Glu) is the predominant excitatory neurotransmitter in the mammalian central nervous system. It plays major roles in normal neurophysiology and many brain disorders by binding to membrane-bound Glu receptors. To overcome the spatial and temporal limitations encountered in previous in vivo extracellular Gl...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Day BK,Pomerleau F,Burmeister JJ,Huettl P,Gerhardt GA

    更新日期:2006-03-01 00:00:00

  • Retinoids and glucocorticoids target common genes in hippocampal HT22 cells.

    abstract::Vitamin A metabolite retinoic acid (RA) plays a major role in the aging adult brain plasticity. Conversely, chronic excess of glucocorticoids (GC) elicits some deleterious effects in the hippocampus. We questioned here the involvement of RA and GC in the expression of target proteins in hippocampal neurons. We investi...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Brossaud J,Roumes H,Moisan MP,Pallet V,Redonnet A,Corcuff JB

    更新日期:2013-05-01 00:00:00

  • Schwann cell marker defined by a monoclonal antibody (224-58) with species cross-reactivity. I. Cellular localization.

    abstract::We have demonstrated by indirect immunofluorescence the cellular localization of a monoclonal antibody (mAb 224-58), produced after immunization of a mouse with human central nervous system (CNS) myelin. Serologically, mAb 224-58 was found to be specific for 3'-sulfomonogalactosylglycolipids, namely 3'-sulfogalactosyl...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Guerci A,Monge M,Baron-Van Evercooren A,Lubetzki C,Dancea S,Boutry JM,Goujet-Zalc C,Zalc B

    更新日期:1986-02-01 00:00:00

  • Leptin induces interleukin-1beta release from rat microglial cells through a caspase 1 independent mechanism.

    abstract::Leptin regulates energy balance by suppressing appetite and increasing energy expenditure through actions in the hypothalamus. Recently we demonstrated that the effects of leptin are, at least in part, mediated by the release of interleukin (IL)-1beta in the brain. Microglia constitute the major source of IL-1beta in ...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Pinteaux E,Inoue W,Schmidt L,Molina-Holgado F,Rothwell NJ,Luheshi GN

    更新日期:2007-08-01 00:00:00

  • Rapid determination of norepinephrine, dopamine, serotonin, their precursor amino acids, and related metabolites in discrete brain areas of mice within ten minutes by HPLC with electrochemical detection.

    abstract::A rapid and simple chromatographic procedure using HPLC with electrochemical detection is described for simultaneous determination of the substrates from precursor amino acids to metabolites related to synthesis and metabolism of three monoamine neurotransmitters--norepinephrine (NE), dopamine (DA), and 5-hydroxytrypt...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Murai S,Saito H,Masuda Y,Itoh T

    更新日期:1988-02-01 00:00:00

  • Lysophosphatidic acid-induced proliferation-related signals in astrocytes.

    abstract::Lysophosphatidic acid (LPA) is a potent lipid biomediator that is likely to have diverse roles in the brain. Thus, LPA-induced events in astrocytes were defined. As little as 1 nM LPA induced a rapid increase in the concentration of intracellular free calcium ([Ca2+]i) in astrocytes from neonatal rat brains. This incr...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Keller JN,Steiner MR,Holtsberg FW,Mattson MP,Steiner SM

    更新日期:1997-09-01 00:00:00

  • Expression of protein phosphatase 2A mutants and silencing of the regulatory B alpha subunit induce a selective loss of acetylated and detyrosinated microtubules.

    abstract::Carboxymethylation and phosphorylation of protein phosphatase 2A (PP2A) catalytic C subunit are evolutionary conserved mechanisms that critically control PP2A holoenzyme assembly and substrate specificity. Down-regulation of PP2A methylation and PP2A enzymes containing the B alpha regulatory subunit occur in Alzheimer...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Nunbhakdi-Craig V,Schuechner S,Sontag JM,Montgomery L,Pallas DC,Juno C,Mudrak I,Ogris E,Sontag E

    更新日期:2007-05-01 00:00:00

  • Glutamine efflux from astrocytes is mediated by multiple pathways.

    abstract::The neurotransmitter glutamate, once released into the synaptic cleft, is largely recycled by the glutamate-glutamine cycle, which involves uptake into astrocytes, conversion into glutamine and subsequent release of glutamine from astrocytes as a precursor for neuroneal glutamate synthesis. We analysed glutamine efflu...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Deitmer JW,Bröer A,Bröer S

    更新日期:2003-10-01 00:00:00

  • Transport of prostaglandins and other eicosanoids by the choroid plexus: its characterization and physiological significance.

    abstract::Choroid plexi from the lateral ventricles of rabbits, cats, and dogfish (Mustelus canis) were used to characterize the prostaglandin (PG) uptake process and to establish its kinetic parameters and substrate specificity. The apparent Kt for PGF2 alpha transport by the rabbit choroid plexus was 20 microM; the Jmax was 2...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: DiBenedetto FE,Bito LZ

    更新日期:1986-06-01 00:00:00

  • IMP-GMP 5'-nucleotidase from rat brain: activation by polyphosphates.

    abstract::IMP-GMP 5'-nucleotidase has been purified to homogeneity from total rat brain extracts. This preparation showed a unique band (Mr 54,000 +/- 1,509) in sodium dodecyl sulfate-polyacrylamide gel electrophoresis. The enzyme presented the following properties: optimal pH value, 6.5-6.8; relative velocity measured in the p...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Marques AF,Teixeira NA,Gambaretto C,Sillero A,Sillero MA

    更新日期:1998-09-01 00:00:00

  • Sequence of guinea pig myelin basic protein.

    abstract::This paper proposes a tentative amino acid sequence of guinea pig myelin basic protein obtained by comparison of peptide fragments of the guinea pig and bovine proteins. Analyses of the tryptic peptides confirmed the known sequence differences in the NH2-terminal half of the molecule and showed that in the COOH-termin...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Deibler GE,Martenson RE,Krutzsch HC,Kies MW

    更新日期:1984-07-01 00:00:00

  • Glutamate inhibition of the adrenergic-stimulated production of melatonin in rat pineal gland in vitro.

    abstract::The effect of L-glutamate on the adrenergic-stimulated release of melatonin in the rat pineal gland was examined using an in vitro perfusion system. L-Glutamate by itself had no effect on melatonin secretion whereas L-glutamate administered prior to (-)-isoproterenol (beta-adrenergic agonist) and L-phenylephrine (alph...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Kus L,Handa RJ,McNulty JA

    更新日期:1994-06-01 00:00:00

  • Ontogenesis of alpha 2-adrenoceptor coupling with GTP-binding proteins in the rat telencephalon.

    abstract::The ontogenesis of alpha 2-adrenoceptors and GTP-binding proteins and their coupling activity were investigated in telencephalon membranes of developing rats. The manganese-induced elevation of [3H]clonidine binding was increased in an age-dependent manner but the guanosine 5'-O-(3-thio)triphosphate-induced decrease i...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Kitamura Y,Mochii M,Kodama R,Agata K,Watanabe K,Eguchi G,Nomura Y

    更新日期:1989-07-01 00:00:00

  • Cerebrovascular endothelial cell culture: metabolism and synthesis of 5-hydroxytryptamine.

    abstract::The presence of 5-hydroxytryptamine was investigated in cultured and propagated cerebrovascular endothelium using immunohistochemistry and high pressure liquid chromatography. These studies demonstrate that the endothelium has the ability to take up and metabolize 5-hydroxytryptamine as well as to synthesize this amin...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Maruki C,Spatz M,Ueki Y,Nagatsu I,Bembry J

    更新日期:1984-08-01 00:00:00

  • Nicastrin is required for amyloid precursor protein (APP) but not Notch processing, while anterior pharynx-defective 1 is dispensable for processing of both APP and Notch.

    abstract::The γ-secretase complex is composed of at least four components: presenilin 1 or presenilin-2, nicastrin (NCT), anterior pharynx-defective 1 (Aph-1), and presenilin enhancer 2. In this study, using knockout cell lines, our data demonstrated that knockout of NCT, as well as knockout of presenilin enhancer 2, completely...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Hu C,Zeng L,Li T,Meyer MA,Cui MZ,Xu X

    更新日期:2016-03-01 00:00:00

  • Altered patterns of protein phosphorylation and synthesis caused by methyl mercury in cerebellar granule cell culture.

    abstract::In the preceding report we demonstrated a dose-dependent increase in 32P-phosphoprotein labeling after 24-h exposure of cultured cerebellar granule neurons to methyl mercury (MeHg), a response that was not observed in glial cultures. In the present study we have examined 32P-labeled phosphoproteins by two-dimensional ...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Sarafian T,Verity MA

    更新日期:1990-09-01 00:00:00

  • Intragenic sequences affect the expression of the gene encoding glial fibrillary acidic protein.

    abstract::We show that the expression of the gene encoding glial fibrillary acidic protein (GFAP) gene is affected by at least three cis-acting elements. A positive regulatory element that is located between nucleotides -1,631 and -1,479 can confer cell type-specific expression on a heterologous gene. A second regulatory elemen...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Sarkar S,Cowan NJ

    更新日期:1991-08-01 00:00:00

  • Purification and properties of succinyl-CoA:3-oxo-acid CoA-transferase from rat brain.

    abstract::Rat brain succinyl-CoA:3-oxo-acid CoA-transferase (3-oxo-acid CoA-transferase, EC, the first committed enzyme in the oxidation of ketone bodies in mitochondria, was purified to apparent homogeneity as judged by polyacrylamide gel electrophoresis. The enzyme has an apparent molecular weight of 90,000 as determ...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Russell JJ,Patel MS

    更新日期:1982-05-01 00:00:00

  • Artifactual high-affinity and saturable binding of [3H]5-hydroxytryptamine induced by radioligand oxidation.

    abstract::The binding of [3H]5-hydroxytryptamine (5-HT, serotonin) to cerebellar membranes was examined after preincubation of [3H]5-HT in the presence or absence of ascorbate. The tissue preparation was identical in all experiments and consisted of rat cerebellar homogenates in Tris-HCl buffer with 0.1% ascorbate. Cerebellar m...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Peroutka SJ,Ison PJ,Liu DU,Barrett RW

    更新日期:1986-07-01 00:00:00

  • Changes in nine enzyme markers for neurons, glia, and endothelial cells in agonal state and Huntington's disease caudate nucleus.

    abstract::Enzymes considered to be markers for neurons (angiotensin converting enzyme, thermolysin-like metalloendopeptidase, alanine aminopeptidase, and glutamate-oxaloacetate transaminase), glia (glutamine synthetase, pyruvate carboxylase, and beta-glucuronidase), and endothelial cells (alkaline phosphatase and plasminogen ac...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Butterworth J

    更新日期:1986-08-01 00:00:00

  • A one-step procedure for isolation of poly(A)+ mRNA from isolated brain capillaries and endothelial cells in culture.

    abstract::The study of the regulation of low-abundance blood-brain barrier (BBB) transcripts either in isolated brain microvessels or in endothelial cells in tissue culture (ECL cells) requires isolation of poly(A)+ mRNA. Therefore, we describe here a single-step method for isolation of poly(A)+ mRNA from brain capillaries or E...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Boado RJ,Pardridge MM

    更新日期:1991-12-01 00:00:00

  • IFN-gamma and IL-4 differentially shape metabolic responses and neuroprotective phenotype of astrocytes.

    abstract::Astrocytes can either exacerbate or ameliorate secondary degeneration at sites of injury in the CNS but the contextual basis for eliciting these opposing phenotypes is poorly understood. In this study, we demonstrate that the two major cytokines produced by Th1 and Th2 cells, interferon-gamma (IFN-gamma), and interleu...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Garg SK,Kipnis J,Banerjee R

    更新日期:2009-03-01 00:00:00

  • Glutamate activates GFAP gene promoter from cultured astrocytes through TGF-beta1 pathways.

    abstract::Glial cells are currently viewed as active partners of neurons in synapse formation. The close proximity of astrocytes to the synaptic cleft suggests that these cells might be potential targets for neuronal-released molecules although this issue has been less addressed. Here, we evaluated the role of the excitatory ne...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Romão LF,Sousa Vde O,Neto VM,Gomes FC

    更新日期:2008-07-01 00:00:00

  • [3H]8-hydroxy-2-(di-n-propylamino)tetralin binding to pre- and postsynaptic 5-hydroxytryptamine sites in various regions of the rat brain.

    abstract::The specific binding of [3H]8-hydroxy-2-(di-n-propylamino)tetralin ([ 3H]8-OH-DPAT) to 5-hydroxytryptamine (5-HT)-related sites was investigated in several regions of the rat brain. Marked differences were observed in the characteristics of binding to membranes from hippocampus, striatum, and cerebral cortex. Hippocam...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Hall MD,el Mestikawy S,Emerit MB,Pichat L,Hamon M,Gozlan H

    更新日期:1985-06-01 00:00:00

  • The mechanism of membrane-translocation of regulator of G-protein signaling (RGS) 8 induced by Galpha expression.

    abstract::RGS (regulators of G-protein signaling) proteins comprise a large family that modulates heterotrimeric G-protein signaling. This protein family has a common RGS domain and functions as GTPase-activating proteins for the alpha-subunits of heterotrimeric G-proteins located at the plasma membrane. RGS8 was identified as ...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Masuho I,Itoh M,Itoh H,Saitoh O

    更新日期:2004-01-01 00:00:00

  • Effects of different semipermeable membranes on in vitro and in vivo performance of microdialysis probes.

    abstract::The in vitro and in vivo performance of three different semipermeable microdialysis membranes was compared: a proprietary polycarbonate-ether membrane made by Carnegie Medecin; cuprophan, a regenerated cellulose membrane; and polyacrylonitrile. When microdialysis probes were tested in a stirred in vitro solution, larg...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Hsiao JK,Ball BA,Morrison PF,Mefford IN,Bungay PM

    更新日期:1990-04-01 00:00:00

  • GT3 synthesis in the proximal Golgi occurs in a compartment different from those for GD3 and GM3 synthesis.

    abstract::Previous studies from this laboratory have shown that synthesis of GT3, the precursor of c series gangliosides, occurs in proximal Golgi compartments, as has been shown for the synthesis of GM3 and GD3, the precursors of a and b series gangliosides, respectively. In this work we studied whether the synthesis of GM3, G...

    journal_title:Journal of neurochemistry

    pub_type: 杂志文章


    authors: Rosales Fritz VM,Maxzúd MK,Maccioni HJ

    更新日期:1996-10-01 00:00:00