Neuroinflammatory and oxidative stress phenomena in MPS IIIA mouse model: the positive effect of long-term aspirin treatment.


:Sanfilippo disease (MPS IIIA) is an autosomal recessive lysosomal storage disorder resulting from sulfamidase deficiency, which is characterized by severe neurological impairment. Various tissues of MPS IIIA mice accumulate undegraded glycosaminoglycans and mimic the human neurodegenerative disorder, and are an excellent tool to both delineate disease pathogenesis and test potential therapies. The relationship between abnormal glycosaminoglycan storage and neurodysfunction remains ill defined. Pathways such as inflammation or oxidative stress have been highlighted in many neurodegenerative disorders, including lysosomal storage diseases, as major components of the neuropathology. By using quantitative polymerase chain reaction, we have compared the expression of selected genes in normal and MPS IIIA mouse cerebral tissues, focusing on inflammation, apoptosis and oxidative stress-related genes. We have identified several genes strongly over-expressed in the central nervous system of a MPS IIIA mouse, reflecting a neurological deterioration state. We have used these genes as markers to follow-up a long-term aspirin treatment. Aspirin treatment led to the normalization of inflammation- and oxidative stress-related mRNA levels in treated MPS IIIA mouse brains. A biochemical correction of an oxidative stress phenomenon both in the brain and peripheral organs of treated MPS IIIA mice was also obtained. These results suggest that anti-inflammatory intervention may be of potential benefit in MPS IIIA disease.


Mol Genet Metab


Arfi A,Richard M,Gandolphe C,Bonnefont-Rousselot D,Thérond P,Scherman D




Has Abstract


2011-05-01 00:00:00














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    pub_type: 杂志文章


    authors: Daghman NA,McHale CM,Savage GM,Price S,Winter PC,Maxwell AP,Lappin TR

    更新日期:1999-06-01 00:00:00

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    authors: Tayebi N,Walker J,Stubblefield B,Orvisky E,LaMarca ME,Wong K,Rosenbaum H,Schiffmann R,Bembi B,Sidransky E

    更新日期:2003-06-01 00:00:00

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    authors: Rocha JC,van Spronsen FJ,Almeida MF,Soares G,Quelhas D,Ramos E,Guimarães JT,Borges N

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    authors: Berger I,Ben-Neriah Z,Dor-Wolman T,Shaag A,Saada A,Zenvirt S,Raas-Rothschild A,Nadjari M,Kaestner KH,Elpeleg O

    更新日期:2011-12-01 00:00:00

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    pub_type: 杂志文章


    authors: Brautbar A,Wang J,Abdenur JE,Chang RC,Thomas JA,Grebe TA,Lim C,Weng SW,Graham BH,Wong LJ

    更新日期:2008-08-01 00:00:00

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    pub_type: 杂志文章


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    更新日期:2006-04-01 00:00:00

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    pub_type: 杂志文章,评审


    authors: Paupe V,Gilbert T,Le Merrer M,Munnich A,Cormier-Daire V,El Ghouzzi V

    更新日期:2004-09-01 00:00:00

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    pub_type: 杂志文章


    authors: Karim MA,Craig RL,Wang X,Hale TC,Elbein SC

    更新日期:2006-06-01 00:00:00

  • Homomeric and heteromeric interactions between wild-type and mutant phenylalanine hydroxylase subunits: evaluation of two-hybrid approaches for functional analysis of mutations causing hyperphenylalaninemia.

    abstract::Phenylketonuria (PKU) is caused by mutations in the phenylalanine hydroxylase gene (PAH), while mutations in genes encoding the two enzymes (dihydropteridine reductase, DHPR, and pterin-4-alpha-carbinolamine dehydratase, PCD) required for recycling of its cofactor, tetrahydrobiopterin (BH(4)), cause other rarer diseas...

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    pub_type: 杂志文章


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    更新日期:2001-07-01 00:00:00

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    pub_type: 杂志文章


    authors: Sato Y,Kobayashi H,Sato S,Shimada Y,Fukuda T,Eto Y,Ohashi T,Ida H

    更新日期:2014-07-01 00:00:00

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    pub_type: 杂志文章,评审


    authors: Sarkissian CN,Gámez A

    更新日期:2005-12-01 00:00:00

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    更新日期:2007-09-01 00:00:00

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    pub_type: 杂志文章,评审


    authors: Suopanki J,Partanen S,Ezaki J,Baumann M,Kominami E,Tyynelä J

    更新日期:2000-09-01 00:00:00

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    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章


    authors: Lin Z,Suzow JG,Fontaine JM,Naylor EW

    更新日期:2004-03-01 00:00:00

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    authors: Oladipo OO,Weindel AL,Saunders AN,Dietzen DJ

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    pub_type: 杂志文章


    authors: O'Brien KJ,Lozier J,Cullinane AR,Osorio B,Nghiem K,Speransky V,Zein WM,Mullikin JC,Neff AT,Simon KL,Malicdan MC,Gahl WA,Young LR,Gochuico BR

    更新日期:2016-11-01 00:00:00

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    authors: Teresa E,Lonardo F,Fiumara A,Lombardi C,Russo P,Zuppi C,Scarano G,Musumeci S,Gianfrancesco F

    更新日期:2006-06-01 00:00:00

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    authors: Maor SB,Abramovitch S,Erdos MR,Brody LC,Werner H

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    authors: Babajani G,Tropak MB,Mahuran DJ,Kermode AR

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    authors: Richards DY,Winn SR,Dudley S,Fedorov L,Rimann N,Thöny B,Harding CO

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    authors: Hall P,Michels V,Gavrilov D,Matern D,Oglesbee D,Raymond K,Rinaldo P,Tortorelli S

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    pub_type: 杂志文章


    authors: Falk MJ,Zhang Z,Rosenjack JR,Nissim I,Daikhin E,Nissim I,Sedensky MM,Yudkoff M,Morgan PG

    更新日期:2008-04-01 00:00:00

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    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章


    authors: Salazar DA,Rodríguez-López A,Herreño A,Barbosa H,Herrera J,Ardila A,Barreto GE,González J,Alméciga-Díaz CJ

    更新日期:2016-02-01 00:00:00

  • Stop codon read-through of a methylmalonic aciduria mutation.

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    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章


    authors: Buck NE,Wood L,Hu R,Peters HL

    更新日期:2009-08-01 00:00:00

  • Combined Hurler and Sanfilippo syndrome in a sibling pair.

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    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章


    authors: Sun A,Hopwood JJ,Thompson J,Cederbaum SD

    更新日期:2011-06-01 00:00:00

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    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章


    authors: Lin HY,Chuang CK,Chen MR,Lin SM,Hung CL,Chang CY,Chiu PC,Tsai WH,Niu DM,Tsai FJ,Lin SJ,Hwu WL,Lin JL,Lin SP

    更新日期:2016-04-01 00:00:00

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    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章


    authors: Grünert SC,Schmitt RN,Schlatter SM,Gemperle-Britschgi C,Balcı MC,Berg V,Çoker M,Das AM,Demirkol M,Derks TGJ,Gökçay G,Uçar SK,Konstantopoulou V,Christoph Korenke G,Lotz-Havla AS,Schlune A,Staufner C,Tran C,Visser G,S

    更新日期:2017-09-01 00:00:00

  • ELMO1 variants and susceptibility to diabetic nephropathy in American Indians.

    abstract::Variants in the engulfment and cell motility 1 gene, ELMO1, have previously been associated with kidney disease attributed to type 2 diabetes. The Pima Indians of Arizona have high rates of diabetic nephropathy, which is strongly dependent on genetic determinants; thus, we sought to investigate the role of ELMO1 polym...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章


    authors: Hanson RL,Millis MP,Young NJ,Kobes S,Nelson RG,Knowler WC,DiStefano JK

    更新日期:2010-12-01 00:00:00