RNAi-mediated silencing of ABCD3 gene expression in rat C6 glial cells: a model system to study PMP70 function.


:The function of PMP70, one of the four ABC half-transporters of mammalian peroxisomes, encoded by ABCD3 gene, is still unclear. The finding that PMP70 over-expression partially corrected very long-chain fatty acid oxidation defects in fibroblasts of X-linked adrenoleukodystrophy patients, has unveiled its potential clinical relevance, prompting us to set up a model system to study PMP70 function. We used the RNA interference technique, a powerful approach to loss-of-function gene expression analysis, to knockdown the ABCD3 gene in the rat glial C6 cell line, since glia could represent the target tissue of X-linked adrenoleukodystrophy disease. Cells were transfected with a vector for RNA interference generating small interfering RNAs that specifically target the ABCD3 mRNA. By using a puromycin-selectable version of the plasmid, we generated a stable cell line (abcd3kd), in which we observed a stable decrease of PMP70 protein expression greater than 70%. We thus examined the effect of ABCD3 knockdown on lignoceric and palmitic acids beta-oxidation and we found that in abcd3kd cells the rate of peroxisomal and mitochondrial beta-oxidation activities were both reduced about one-third compared with control cells. The mitochondrial membrane potential, determined by cytofluorometric analysis, was also affected. Lipid and fatty acid analyses of abcd3kd cells showed an increase of hexacosenoic acid (C26:0) in the cholesteryl-ester fraction. These results add another clue about the overlapping function of PMP70 and ALDP, the peroxisomal protein involved in X-linked adrenoleukodystrophy, since C26:0 is the biochemical marker of the disease and in the brain lesions it is accumulated in the cholesteryl-ester fraction. Considered as a whole, our results indicate that the abcd3kd cell line is a valuable tool to further study the function of PMP70 and eventually its role in X-linked adrenoleukodystrophy.


Neurochem Int


Di Benedetto R,Denti MA,Salvati S,Sanchez M,Attorri L,David G,Di Biase A




Has Abstract


2008-05-01 00:00:00














  • In vitro changes of ganglioside immunostain localization in developing neural cells.

    abstract::The immunostains of GD3, c-pathway polysialogangliosides and gangliotetraosylgangliosides belonging to the a- and b-pathways were analyzed in embryo optic lobe cells. Cells were cultured in serum free media with, and without, bovine brain ganglioside mixture (largely gangliotetraosylgangliosides). Control cells differ...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Cossy-Isasi S,Panzetta P

    更新日期:1994-04-01 00:00:00

  • Differential effect of platelet activating factor on 1-methyl-4-phenylpyridinium-induced cell death through regulation of apoptosis-related protein activation.

    abstract::Platelet activating factor (PAF) has been suggested to play a critical role in the pathogenesis of neurological disorders. We assessed the effect of PAF against the toxicity of 1-methyl-4-phenylpyridinium (MPP(+)), a parkinsonian toxin, in relation to apoptotic process. PAF exhibited differential effect against the MP...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Ha SW,Lee CS

    更新日期:2010-05-01 00:00:00

  • Effect of calmodulin antagonists on phospholipase D activity in SH-SY5Y cells.

    abstract::The aim of this study was to investigate the involvement of calmodulin in phospholipase D activation in SH-SY5Y cells. Cells prelabelled with [3H]-palmitic acid were incubated with calmodulin antagonists and/or other compounds. Phosphatidylethanol, a specific marker for phospholipase D activity, and phosphatidic acid ...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: del Carmen Boyano-Adánez M,Gustavsson L

    更新日期:2002-03-01 00:00:00

  • Therapeutic hypothermia protects photoreceptors through activating Cirbp pathway.

    abstract::Therapeutic hypothermia as a physical method to lower the brain temperature of patients has been widely used in clinics as an effective and necessary step during the treatment of acute brain injury or edema. However, due to limitations of the ocular structure, the application of hypothermia in retinal neuroprotection ...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Sun YJ,Ma S,Fan B,Wang Y,Wang SR,Li GY

    更新日期:2019-06-01 00:00:00

  • Neuroprotection with the proteasome inhibitor MLN519 in focal ischemic brain injury: relation to nuclear factor kappaB (NF-kappaB), inflammatory gene expression, and leukocyte infiltration.

    abstract::The ubiquitin proteasome system (UPS) is a major cellular protein degradation pathway that involves the modulation of key proteins controlling inflammation, cell cycle regulation and gene expression. Modulation of the UPS with proteasome inhibitors has indicated efficacy in the treatment of several disease states incl...

    journal_title:Neurochemistry international

    pub_type: 杂志文章,评审


    authors: Williams AJ,Dave JR,Tortella FC

    更新日期:2006-07-01 00:00:00

  • Midbrain and forebrain patterning delivers immunocytochemically and functionally similar populations of neuropeptide Y containing GABAergic neurons.

    abstract::Neurons differentiated in vitro from embryonic stem cells (ESCs) have the potential to serve both as models of disease states and in drug discovery programs. In this study, we use sonic hedgehog (SHH) and fibroblast growth factor 8 (FGF-8) to enrich for forebrain and midbrain phenotypes from mouse ESCs. We then invest...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Khaira SK,Nefzger CM,Beh SJ,Pouton CW,Haynes JM

    更新日期:2011-09-01 00:00:00

  • Effects of periodate oxidation and glycosidases on structural and functional properties of the acetylcholine receptor and the non-receptor, peripheral ?-polypeptide (M(r) 43,000).

    abstract::NaIO(4) oxidation, exo- and endo-glycosidase treatments and combinations thereof have been applied to acetylcholine receptor from Torpedo marmorata in its membrane-bound and detergent-solubilised forms. The effects of these chemical and enzymatic treatments are made apparent in the electrophoretic properties of the fo...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Criado M,Barrantes FJ

    更新日期:1982-01-01 00:00:00

  • An hypothesis for a vitamin A cycle in the pigment epithelium of bovine retina.

    abstract::Several metabolic transformations of vitamin A have now been found in the pigment epithelium of cattle retina, leading to the hypothesis that there may be a vitamin A cycle in these cells. A retinol binding protein of MW ? 17,000 in pigment epithelial cytosol has been identified independently in several laboratories. ...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Berman ER,Segal N,Schneider A,Feeney L

    更新日期:1980-01-01 00:00:00

  • Lectins as probes of glycoprotein and glycolipid oligosaccharides in rods and cones.

    abstract::The oligosaccharides of rod and cone membranes were investigated with the aid of fluorescence and (125)I-labeled lectins. Additionally, the ability of lectins to cause agglutination in ROS suspensions was used as an index for the presence of the corresponding lectin receptors. The specificities of lectin-ligand intera...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Bridges CD,Fong SL

    更新日期:1980-01-01 00:00:00

  • 25-Hydroxycholesterol causes death but does not prevent nerve growth factor-induced neurite outgrowth in PC12 cells.

    abstract::The effects of cholesterol oxides on regular and neuronal PC12 cells were examined. Among the cholesterol oxides tested, 7-beta-, 22(R)- and 25-OH-cholesterol were very toxic to regular PC12 cells such that approximately 80% of cells were killed after a 3 day incubation with 20 micrograms/ml (50 microM) of these agent...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Chang JY,Liu LZ

    更新日期:1997-10-01 00:00:00

  • Effects of chronic variate stress on feeding behavior and on monoamine levels in different rat brain structures.

    abstract::Chronic variate stress was seen to decrease the ingestion of sweet food when compared to control rats. Brain monoamines are known to be involved in the control of food intake, serotonin appears to be involved in the mechanisms of satiety, and dopamine in mediating appetite or approach behaviors triggered by incentive ...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Gamaro GD,Manoli LP,Torres IL,Silveira R,Dalmaz C

    更新日期:2003-01-01 00:00:00

  • Beta-amyloid and brain-derived neurotrophic factor, BDNF, up-regulate the expression of glutamate transporter GLT-1/EAAT2 via different signaling pathways utilizing transcription factor NF-kappaB.

    abstract::Malfunctioning of high-affinity glutamate transporters is believed to contribute to the accumulation of toxic concentrations of glutamate and, thus, trigger the cellular mechanisms of neurodegeneration. Emerging data point to the presence of excitotoxic component in Alzheimer's disease (AD) and aberrant expression of ...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Rodriguez-Kern A,Gegelashvili M,Schousboe A,Zhang J,Sung L,Gegelashvili G

    更新日期:2003-09-01 00:00:00

  • Identification of two proteolipid antigens with different localization at torpedo neuromuscular synapses.

    abstract::The cholinergic nerve terminals of Torpedo electric organ are a rich source of a hydrophobic protein, the mediatophore, which endows artificial membranes with a calcium dependent mechanism that translocates acetylcholine. An antiserum raised against purified mediatophore permitted the identification of two different p...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Morel N,Israel M,Synguelakis M,Le Gal la Salle G

    更新日期:1989-01-01 00:00:00

  • Downregulation of Mfn2 participates in manganese-induced neuronal apoptosis in rat striatum and PC12 cells.

    abstract::Manganese (Mn) is a widely distributed trace element that is essential for normal brain function and development. However, chronic exposure to excessive Mn has been known to lead to neuronal loss and manganism, a disease with debilitating motor and cognitive deficits, whose clinical syndrome resembling idiopathic Park...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Liu X,Yang J,Lu C,Jiang S,Nie X,Han J,Yin L,Jiang J

    更新日期:2017-09-01 00:00:00

  • High-affinity choline uptake and acetylcholine-metabolizing enzymes in CNS white matter. A quantitative study.

    abstract::The presence of nicotinic and muscarinic receptors suggests the occurrence of cholinergic neurotransmission in white matter; however no quantitative information exists on acetylcholine formation and breakdown in white matter. We compared white structures of pig brain (fimbria, corpus callosum, pyramidal tracts, and oc...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Hassel B,Solyga V,Lossius A

    更新日期:2008-12-01 00:00:00

  • Enhancement of serotonin transporter function by tumor necrosis factor alpha but not by interleukin-6.

    abstract::Serotonin (5-HT) is a prime candidate for studies of the interaction between the nervous and immune systems, since it is both an important neurotransmitter and released at high concentrations at sites of inflammation. Serotonergic neurotransmission is regulated by the 5-HT transporter (5-HTT), which determines the mag...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Mössner R,Heils A,Stöber G,Okladnova O,Daniel S,Lesch KP

    更新日期:1998-09-01 00:00:00

  • Comparison of [(3)H]-(2S,4R)-4-methylglutamate and [(3)H]D-aspartate as ligands for binding and autoradiographic analyses of glutamate transporters.

    abstract::While studies with [(3)H]D-aspartate ([(3)H]d-Asp) illustrate specific interactions with excitatory amino acid transporters (EAATs), new insights into the pharmacological characteristics and localization of specific EAAT subtypes depend upon the availability of novel ligands. One such ligand is [(3)H]-(2S,4R)-4-methyl...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Apricò K,Beart PM,Crawford D,O'shea RD

    更新日期:2007-12-01 00:00:00

  • Direct demonstration by [13C]NMR spectroscopy that glutamine from astrocytes is a precursor for GABA synthesis in neurons.

    abstract::Primary cultures of cerebral cortical astrocytes and neurons, as well as neurons growing on top of the astrocytes (sandwich co-cultures), were incubated with 1-[13C]glucose or 2-[13C]acetate and in the presence or absence of the glutamine synthetase inhibitor methionine sulfoximine. [13C]NMR spectroscopy at 125 MHz wa...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Sonnewald U,Westergaard N,Schousboe A,Svendsen JS,Unsgård G,Petersen SB

    更新日期:1993-01-01 00:00:00

  • Insulin-stimulated taurine uptake in rat retina and retinal pigment epithelium.

    abstract::Taurine is found at millimolar concentration in the retina and retinal pigment epithelium. High concentrations of taurine are essential for maintenance of retinal function. Taurine uptake by retina and retinal pigment epithelium was significantly enhanced by physiological concentrations of insulin as well as by high g...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Salceda R

    更新日期:1999-10-01 00:00:00

  • Synthesis and breakdown of different sized retinal proteins in darkness and during photic stimulation.

    abstract::Rabbit retinas were maintained in vitro in medium resembling cerebrospinal fluid and were exposed to (3)H-leucine or (14)C-leucine in double-labeling experiments designed to measure rates of protein turnover and to determine the effects of photic stimulation on protein synthesis and degradation. The retinas were solub...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Ames A 3rd,Parks JM,Nesbett FB

    更新日期:1980-01-01 00:00:00

  • Antidepressant-like effects of 3,6'-disinapoyl sucrose on hippocampal neuronal plasticity and neurotrophic signal pathway in chronically mild stressed rats.

    abstract::Recent studies suggest that the behavioral effects of chronic antidepressant treatment are mediated by stimulation of hippocampal neuronal plasticity and neurogenesis. The present study was designed to examine the effects of 3,6'-disinapoyl sucrose (DISS), a bioactive component of Polygala tenuifolia Willd, on the exp...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Hu Y,Liao HB,Dai-Hong G,Liu P,Wang YY,Rahman K

    更新日期:2010-02-01 00:00:00

  • Activated microglia are the principal glial source of thromboxane in the central nervous system.

    abstract::Thromboxane A2(TxA2) is a potent vasoconstrictor associated with cerebrovascular disease and is thought to be synthesized within tissues of the brain. In order to determine the cellular sources of TxA2 in the central nervous system (CNS), we measured the release of the stable metabolite TxB2 in cultures of mixed or hi...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Giulian D,Corpuz M,Richmond B,Wendt E,Hall ER

    更新日期:1996-07-01 00:00:00

  • Effects of 3-hydroxy-4-amino-4,5,6,7-tetrahydro-1,2-benzisoxazol (exo-THPO) and its N-substituted analogs on GABA transport in cultured neurons and astrocytes and by the four cloned mouse GABA transporters.

    abstract::The system of GABA transporters in neural cells constitutes an efficient mechanism for terminating inhibitory GABAergic neurotransmission. As such these transporter are important therapeutical targets in epilepsy and potentially other neurological diseases related to the GABA system. In this study a number of analogs ...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Sarup A,Larsson OM,Bolvig T,Frølund B,Krogsgaard-Larsen P,Schousboe A

    更新日期:2003-09-01 00:00:00

  • Axonal transports of tripeptidyl peptidase II in rat sciatic nerves.

    abstract::Axonal transport of tripeptidyl peptidase II, a putative cholecystokinin inactivating serine peptidase, was examined in the proximal, middle, and distal segments of rat sciatic nerves using a double ligation technique. Enzyme activity significantly increased not only in the proximal segment but also in the distal segm...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Chikuma T,Shimizu M,Tsuchiya Y,Kato T,Hojo H

    更新日期:2007-01-01 00:00:00

  • The relation between respiratory inhibition and uptake of 1-methyl-isoquinoline (MIQ+) in mitochondria.

    abstract::The effect of 1-methyl-isoquinoline (MIQ+) on the respiratory inhibition and the uptake of MIQ+ were measured using mouse liver mitochondria. MIQ+ inhibited the electron transport of complex I but did not inhibit the respiration of mitochondria with succinate as a substrate. MIQ+ was taken up by mitochondria in an ene...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Aiuchi T,Matsunaga M,Syo M,Nakaya K

    更新日期:1996-03-01 00:00:00

  • New insights into molecular structure and function of ectonucleotidases in the nervous system.

    abstract::Nucleotides can be released as signaling substances in the nervous system from both neural and glial cells. Their function is terminated by ecto-nucleotidases and sequential extracellular metabolism to the nucleoside. Recently considerable progress has been made in unraveling the molecular structure of an ecto-ATPase ...

    journal_title:Neurochemistry international

    pub_type: 杂志文章,评审


    authors: Zimmermann H,Braun N,Kegel B,Heine P

    更新日期:1998-05-01 00:00:00

  • Ammonium induced dysfunction of 5-HT2B receptor in astrocytes.

    abstract::Previously we reported that gene expression of astrocytic 5-HT2B receptors was decreased in brains of depressed animals exposed to chronic mild stress (CMS) (Li et al., 2012) and of Parkinson's disease (Song et al., 2018). Depression is also one of the psychiatric symptoms in hyperammonemia, and astrocyte is a primary...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Yue T,Li B,Gu L,Huang J,Verkhratsky A,Peng L

    更新日期:2019-10-01 00:00:00

  • Serotonin, a potent modulator of arachidonic acid turnover, interaction with glutamatergic receptor in brain cortex.

    abstract::Brain cortex synaptoneurosomes actively incorporated [14C]arachidonic acid (AA) into lipids. Serotonin (5-HT), at a concentration range of 10 microM-1 mM, significantly stimulates the incorporation of AA mainly into phosphatidylinositol (PI) of brain cortex synaptoneurosomes. The stimulation rate of AA incorporation b...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Strosznajder J,Samochocki M,Duran M

    更新日期:1994-08-01 00:00:00

  • Effects of imidazolines on noradrenaline release in brain: an investigation into their relationship to imidazoline, alpha 2 and H3 receptors.

    abstract::The present study was carried out to clarify whether the imidazolines clonidine, moxonidine and cirazoline as well as the guanidine aganodine inhibit noradrenaline release in the rat and rabbit brain via imidazoline receptors, alpha 2-adrenoceptors and/or histamine H3 receptors. Slices or synaptosomes from the rat or ...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Schlicker E,Fink K,Kathmann M,Molderings GJ,Göthert M

    更新日期:1997-01-01 00:00:00

  • Unchanged glutamine synthetase activity and increased NMDA receptor density in epileptic human neocortex: implications for the pathophysiology of epilepsy.

    abstract::We investigated whether alterations in glutamate metabolising glutamine synthetase activity occur in human epileptic neocortex, as shown previously for human epileptic hippocampus [Eid, T., Thomas, M.J., Spencer, D.D., Rundén-Pran, E., Lai, J.C.K., Malthankar, G.V., Kim, J.H., Danbolt, N.C., Ottersen, O.P., de Lanerol...

    journal_title:Neurochemistry international

    pub_type: 杂志文章


    authors: Steffens M,Huppertz HJ,Zentner J,Chauzit E,Feuerstein TJ

    更新日期:2005-11-01 00:00:00