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2. Neuroinflammation, mitochondrial defects and neurodegeneration in mucopolysaccharidosis III type C mouse model

Author

Martins, Carla, Hůlková, Helena, Dridi, Larbi, Dormoy-Raclet, Virginie, Grigoryeva, Lubov, Choi, Yoo, Langford-Smith, Alexander, Wilkinson, Fiona L., Ohmi, Kazuhiro, DiCristo, Graziella, Hamel, Edith, Ausseil, Jerôme, Cheillan, David, Moreau, Alain, Svobodová, Eva, Hájková, Zuzana, Tesařová, Markéta, Hansíková, Hana, Bigger, Brian W., Hrebícek, Martin, and Pshezhetsky, Alexey V.

Published
2015
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5. Back Cover, Volume 40, Issue 8

Author

Martins, Carla, primary, de Medeiros, Paula Frassinetti V., additional, Leistner-Segal, Sandra, additional, Dridi, Larbi, additional, Elcioglu, Nursel, additional, Wood, Jill, additional, Behnam, Mahdiyeh, additional, Noyan, Bilge, additional, Lacerda, Lucia, additional, Geraghty, Michael T., additional, Labuda, Damian, additional, Giugliani, Roberto, additional, and Pshezhetsky, Alexey V., additional

Published
2019
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6. Molecular characterization of a large group of Mucopolysaccharidosis type IIIC patients reveals the evolutionary history of the disease

Author

Martins, Carla, primary, de Medeiros, Paula Frassinetti V., additional, Leistner-Segal, Sandra, additional, Dridi, Larbi, additional, Elcioglu, Nursel, additional, Wood, Jill, additional, Behnam, Mahdiyeh, additional, Noyan, Bilge, additional, Lacerda, Lucia, additional, Geraghty, Michael T., additional, Labuda, Damian, additional, Giugliani, Roberto, additional, and Pshezhetsky, Alexey V., additional

Published
2019
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7. A novel adeno-associated virus capsid with enhanced neurotropism corrects a lysosomal transmembrane enzyme deficiency

Author

Tordo, Julie, primary, O’Leary, Claire, additional, Antunes, André S L M, additional, Palomar, Nuria, additional, Aldrin-Kirk, Patrick, additional, Basche, Mark, additional, Bennett, Antonette, additional, D’Souza, Zelpha, additional, Gleitz, Hélène, additional, Godwin, Annie, additional, Holley, Rebecca J, additional, Parker, Helen, additional, Liao, Ai Yin, additional, Rouse, Paul, additional, Youshani, Amir Saam, additional, Dridi, Larbi, additional, Martins, Carla, additional, Levade, Thierry, additional, Stacey, Kevin B, additional, Davis, Daniel M, additional, Dyer, Adam, additional, Clément, Nathalie, additional, Björklund, Tomas, additional, Ali, Robin R, additional, Agbandje-McKenna, Mavis, additional, Rahim, Ahad A, additional, Pshezhetsky, Alexey, additional, Waddington, Simon N, additional, Linden, R Michael, additional, Bigger, Brian W, additional, and Henckaerts, Els, additional

Published
2018
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8. Gene therapy mediated correction of neurological manifestations of MPS IIIC using a novel AAV serotype

Author

O'Leary, Claire, primary, Antunes, Andre, additional, Tordo, Julie, additional, Liao, Ai Yin, additional, Parker, Helen, additional, Gleitz, Helene, additional, Holley, Rebecca, additional, Youshani, Amir Saam, additional, Dridi, Larbi, additional, Martins, Carla, additional, Pshezhetsky, Alexey, additional, Henckearts, Els, additional, and Bigger, Brian, additional

Published
2017
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9. Therapeutic strategies based on modified U1 snRNAs and chaperones for Sanfilippo C splicing mutations

Author

Matos, Liliana, Canals Montferrer, Isaac, Dridi, Larbi, Choi, Yoo, Prata, Maria Joao, Jordan, Peter, Desviat, Lourdes R., Pérez, Belén, Pshezhetsky, Alexey V., Grinberg Vaisman, Daniel Raúl, Alves, Sandra, Vilageliu i Arqués, Lluïsa, and Universitat de Barcelona

Subjects
Medicine(all), Lisosomes, Lysosomal Storage Disease, Mutació (Biologia), Mutation, Genetics(clinical), Pharmacology (medical), Mutation (Biology), Lysosomes, Splicing, Doenças Genéticas
Abstract

Mutations affecting RNA splicing represent more than 20% of the mutant alleles in Sanfilippo syndrome type C, a rare lysosomal storage disorder that causes severe neurodegeneration. Many of these mutations are localized in the conserved donor or acceptor splice sites, while few are found in the nearby nucleotides. METHODS: In this study we tested several therapeutic approaches specifically designed for different splicing mutations depending on how the mutations affect mRNA processing. For three mutations that affect the donor site (c.234 + 1G > A, c.633 + 1G > A and c.1542 + 4dupA), different modified U1 snRNAs recognizing the mutated donor sites, have been developed in an attempt to rescue the normal splicing process. For another mutation that affects an acceptor splice site (c.372-2A > G) and gives rise to a protein lacking four amino acids, a competitive inhibitor of the HGSNAT protein, glucosamine, was tested as a pharmacological chaperone to correct the aberrant folding and to restore the normal trafficking of the protein to the lysosome. RESULTS: Partial correction of c.234 + 1G > A mutation was achieved with a modified U1 snRNA that completely matches the splice donor site suggesting that these molecules may have a therapeutic potential for some splicing mutations. Furthermore, the importance of the splice site sequence context is highlighted as a key factor in the success of this type of therapy. Additionally, glucosamine treatment resulted in an increase in the enzymatic activity, indicating a partial recovery of the correct folding. CONCLUSIONS: We have assayed two therapeutic strategies for different splicing mutations with promising results for the future applications FCT fellowship SFRH/BD/64592/2009 to LM

Published
2014

10. Brain disease in mucopolysaccharidosis III C mouse: Neuroinflammation, mitochondrial defects and neurodegeneration

Author

Pshezhetsky, Alexey, primary, Martins, Carla, additional, Hůlková, Helena, additional, Dridi, Larbi, additional, Grigoryeva, Lubov, additional, Langford-Smith, Alexander, additional, Wilkinson, Fiona, additional, Ohmi, Kazuhiro, additional, Ausseil, Jerôme, additional, Svobodová, Eva Svobodová, additional, Hájková, Zuzana, additional, Tesařová, Markéta, additional, Hansíková, Hana, additional, Bigger, Brian, additional, and Hrebícek, Martin, additional

Published
2015
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11. Therapeutic strategies based on modified U1 snRNAs and chaperones for Sanfilippo C splicing mutations

Author

Matos, Liliana, primary, Canals, Isaac, additional, Dridi, Larbi, additional, Choi, Yoo, additional, Prata, Maria João, additional, Jordan, Peter, additional, Desviat, Lourdes R, additional, Pérez, Belén, additional, Pshezhetsky, Alexey V, additional, Grinberg, Daniel, additional, Alves, Sandra, additional, and Vilageliu, Lluïsa, additional

Published
2014
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13. Mice Doubly-Deficient in Lysosomal Hexosaminidase A and Neuraminidase 4 Show Epileptic Crises and Rapid Neuronal Loss

Author

Seyrantepe, Volkan, primary, Lema, Pablo, additional, Caqueret, Aurore, additional, Dridi, Larbi, additional, Bel Hadj, Samar, additional, Carpentier, Stephane, additional, Boucher, Francine, additional, Levade, Thierry, additional, Carmant, Lionel, additional, Gravel, Roy A., additional, Hamel, Edith, additional, Vachon, Pascal, additional, Di Cristo, Graziella, additional, Michaud, Jacques L., additional, Morales, Carlos R., additional, and Pshezhetsky, Alexey V., additional

Published
2010
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17. gyrAand gyrBMutations Are Implicated in Cross-Resistance to Ciprofloxacin and Moxifloxacin in Clostridium difficile

Author

Dridi, Larbi, Tankovic, Jacques, Burghoffer, Béatrice, Barbut, Frédéric, and Petit, Jean-Claude

Abstract

ABSTRACTA total of 198 nonrepetitive clinical strains of Clostridium difficileisolated from different French hospitals in 1991 (n= 100) and 1997 (n= 98) were screened for decreased susceptibility to fluoroquinolones by plating onto Wilkins-Chalgren agar containing 16 μg of ciprofloxacin per ml. The frequency of decreased susceptibility was 7% (14 of 198) and was identical for the years 1991 and 1997. Serogroups C, H, D, A9, and K accounted for five, four, two, one, and one of the resistant strains, respectively, one strain being nontypeable. Arbitrarily primed PCR typing showed that all resistant strains had unique patterns except two serotype C strains, which could not be clearly distinguished. All isolates with decreased susceptibility carried a mutation either in gyrA(eight mutations, amino acid changes Asp71→Val in one, Thr82→Ile in six, and Ala118→Thr in one) or in gyrB(six mutations, amino acid changes Asp426→Asn in five and Arg447→Leu in one). These changes are similar to those already described in other species except for Asp71→Val, which is novel, and Ala118→Thr, which is exceptional. Attempts to detect the topoisomerase IV parCgene by PCR amplification with universal parCprimers or DNA-DNA hybridization under low-stringency conditions were unsuccessful. The susceptibilities of all resistant strains to ciprofloxacin and ethidium bromide were not affected by the addition of reserpine at 20 μg/ml. In conclusion, decreased susceptibility to fluoroquinolones in C. difficileis rare in France and is associated with the occurrence of a gyrAor gyrBmutation.

Published
2002
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18. Identification de prot?ines de stress chez Lactobacillus delbrueckii bulgaricus par ?lectrophor?se bidimensionnelle

Author

Lim, Eng, Lafon, Anne, Dridi, Larbi, Boudebbouze, Samira, Dusko Ehrlich, S., and Maguin, Emmanuelle

Abstract

Identification of stress proteins in Lactobacillus delbrueckii bulgaricus by two-dimensional electrophoresis. We studied Lactobacillus bulgaricus adaptation to an acid stress. In L. bulgaricus, adapted cells (30 min incubation at pH 4.75) were ?250 fold more tolerant to lethal acid stress (30 min at pH 3.6) than non adapted ones. In order to identify proteins induced during the adaptation, de novo protein synthesis was monitored by 35S methionine labeling of exponentially growing cultures, under standard (pH 6) and adaptive (pH 4.75) conditions. After 2 dimensional (2-D) electrophoresis separation, the protein patterns were compared. At least 30 protein spots showed an increased radioactivity level under acidic conditions. We determined the N-terminal amino acid sequence of 3 highly induced proteins. By comparison to databases, they were identified as the heat shock proteins GroES, GroEL and DnaK. A differential induction was observed for GroES and GroEL, suggesting a post-transcriptionnal regulation mechanism. In this report we established a method for the study of acid stress response in L. bulgaricus. This method will also be useful for further studies on the adaptation of this bacterium to environmental variations. Nous avons ?tudi? l'adaptation de la bact?rie lactique Lactobacillus bulgaricus au stress acide. Nous avons montr? que les bact?ries cultiv?es en milieu "neutre " (pH 6) pr?sentaient une forte l?talit? apr?s transfert dans un milieu acide (choc acide ? pH 3,6). En revanche, les bact?ries pr?alablement adapt?es dans un milieu acide non l?tal (pH 4,75) devenaient ?250 fois plus tol?rantes au choc acide. Apr?s marquage ? la m?thionine 35S et s?paration par ?lectrophor?se bidimensionnelle (2-D), les profils prot?iques obtenus en condition d'adaptation (pH 4,75) et en condition standard (pH 6) sont compar?s. On observe une augmentation significative (> 2) de l'intensit? d'environ 30 spots pendant l'adaptation. La d?termination des s?quences peptidiques N-terminales de 3 prot?ines fortement induites ? pH 4,75 permet d'identifier les prot?ines de choc thermique GroES, GroEL et DnaK. Nous observons des niveaux d'induction diff?rents pour GroES et GroEL, qui pourraient ?tre li?s ? une r?gulation post-transcriptionnelle. Au final, nous avons ?tabli une m?thode d'?tude de la r?ponse au stress acide chez L. bulgaricus. De fa?on plus g?n?rale, cette m?thode permettra d'?tudier l'adaptation de cette bact?rie aux variations environnementales.

Published
2001
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