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1. CRISPR/Cas9-Induced (CTGCAG)n Repeat Instability in the Myotonic Dystrophy Type 1 Locus: Implications for Therapeutic Genome Editing

Author

Agtmaal, E.L. van, Andre, L.M., Willemse, M.P., Cumming, S.A., Kessel, I.D.G. van, Broek, W.J.A.A. van den, Gourdon, G., Furling, D., Mouly, V., Monckton, D.G., Wansink, D.G., Wieringa, B., Agtmaal, E.L. van, Andre, L.M., Willemse, M.P., Cumming, S.A., Kessel, I.D.G. van, Broek, W.J.A.A. van den, Gourdon, G., Furling, D., Mouly, V., Monckton, D.G., Wansink, D.G., and Wieringa, B.

Abstract

Contains fulltext : 174401.pdf (publisher's version ) (Closed access), Myotonic dystrophy type 1 (DM1) is caused by (CTGCAG)n-repeat expansion within the DMPK gene and thought to be mediated by a toxic RNA gain of function. Current attempts to develop therapy for this disease mainly aim at destroying or blocking abnormal properties of mutant DMPK (CUG)n RNA. Here, we explored a DNA-directed strategy and demonstrate that single clustered regularly interspaced short palindromic repeats (CRISPR)/Cas9-cleavage in either its 5' or 3' unique flank promotes uncontrollable deletion of large segments from the expanded trinucleotide repeat, rather than formation of short indels usually seen after double-strand break repair. Complete and precise excision of the repeat tract from normal and large expanded DMPK alleles in myoblasts from unaffected individuals, DM1 patients, and a DM1 mouse model could be achieved at high frequency by dual CRISPR/Cas9-cleavage at either side of the (CTGCAG)n sequence. Importantly, removal of the repeat appeared to have no detrimental effects on the expression of genes in the DM1 locus. Moreover, myogenic capacity, nucleocytoplasmic distribution, and abnormal RNP-binding behavior of transcripts from the edited DMPK gene were normalized. Dual sgRNA-guided excision of the (CTGCAG)n tract by CRISPR/Cas9 technology is applicable for developing isogenic cell lines for research and may provide new therapeutic opportunities for patients with DM1.

Published
2017

2. CRISPR/Cas9-Induced (CTGCAG)n Repeat Instability in the Myotonic Dystrophy Type 1 Locus: Implications for Therapeutic Genome Editing

Author

Agtmaal, E.L. van, Andre, L.M., Willemse, M.P., Cumming, S.A., Kessel, I.D.G. van, Broek, W.J.A.A. van den, Gourdon, G., Furling, D., Mouly, V., Monckton, D.G., Wansink, D.G., Wieringa, B., Agtmaal, E.L. van, Andre, L.M., Willemse, M.P., Cumming, S.A., Kessel, I.D.G. van, Broek, W.J.A.A. van den, Gourdon, G., Furling, D., Mouly, V., Monckton, D.G., Wansink, D.G., and Wieringa, B.

Abstract

Contains fulltext : 174401.pdf (publisher's version ) (Closed access), Myotonic dystrophy type 1 (DM1) is caused by (CTGCAG)n-repeat expansion within the DMPK gene and thought to be mediated by a toxic RNA gain of function. Current attempts to develop therapy for this disease mainly aim at destroying or blocking abnormal properties of mutant DMPK (CUG)n RNA. Here, we explored a DNA-directed strategy and demonstrate that single clustered regularly interspaced short palindromic repeats (CRISPR)/Cas9-cleavage in either its 5' or 3' unique flank promotes uncontrollable deletion of large segments from the expanded trinucleotide repeat, rather than formation of short indels usually seen after double-strand break repair. Complete and precise excision of the repeat tract from normal and large expanded DMPK alleles in myoblasts from unaffected individuals, DM1 patients, and a DM1 mouse model could be achieved at high frequency by dual CRISPR/Cas9-cleavage at either side of the (CTGCAG)n sequence. Importantly, removal of the repeat appeared to have no detrimental effects on the expression of genes in the DM1 locus. Moreover, myogenic capacity, nucleocytoplasmic distribution, and abnormal RNP-binding behavior of transcripts from the edited DMPK gene were normalized. Dual sgRNA-guided excision of the (CTGCAG)n tract by CRISPR/Cas9 technology is applicable for developing isogenic cell lines for research and may provide new therapeutic opportunities for patients with DM1.

Published
2017

3. CRISPR/Cas9-Induced (CTGCAG)n Repeat Instability in the Myotonic Dystrophy Type 1 Locus: Implications for Therapeutic Genome Editing

Author

Agtmaal, E.L. van, Andre, L.M., Willemse, M.P., Cumming, S.A., Kessel, I.D.G. van, Broek, W.J.A.A. van den, Gourdon, G., Furling, D., Mouly, V., Monckton, D.G., Wansink, D.G., Wieringa, B., Agtmaal, E.L. van, Andre, L.M., Willemse, M.P., Cumming, S.A., Kessel, I.D.G. van, Broek, W.J.A.A. van den, Gourdon, G., Furling, D., Mouly, V., Monckton, D.G., Wansink, D.G., and Wieringa, B.

Abstract

Contains fulltext : 174401.pdf (publisher's version ) (Closed access), Myotonic dystrophy type 1 (DM1) is caused by (CTGCAG)n-repeat expansion within the DMPK gene and thought to be mediated by a toxic RNA gain of function. Current attempts to develop therapy for this disease mainly aim at destroying or blocking abnormal properties of mutant DMPK (CUG)n RNA. Here, we explored a DNA-directed strategy and demonstrate that single clustered regularly interspaced short palindromic repeats (CRISPR)/Cas9-cleavage in either its 5' or 3' unique flank promotes uncontrollable deletion of large segments from the expanded trinucleotide repeat, rather than formation of short indels usually seen after double-strand break repair. Complete and precise excision of the repeat tract from normal and large expanded DMPK alleles in myoblasts from unaffected individuals, DM1 patients, and a DM1 mouse model could be achieved at high frequency by dual CRISPR/Cas9-cleavage at either side of the (CTGCAG)n sequence. Importantly, removal of the repeat appeared to have no detrimental effects on the expression of genes in the DM1 locus. Moreover, myogenic capacity, nucleocytoplasmic distribution, and abnormal RNP-binding behavior of transcripts from the edited DMPK gene were normalized. Dual sgRNA-guided excision of the (CTGCAG)n tract by CRISPR/Cas9 technology is applicable for developing isogenic cell lines for research and may provide new therapeutic opportunities for patients with DM1.

Published
2017

4. The mouse mismatch repair protein, MSH3, is a nucleoplasmic protein that aggregates into denser nuclear bodies under conditions of stress.

Author

Holt, I.J., Thanh Lam, L., Tome, S., Wansink, D.G., Riele, H. te, Gourdon, G., Morris, G.E., Holt, I.J., Thanh Lam, L., Tome, S., Wansink, D.G., Riele, H. te, Gourdon, G., and Morris, G.E.

Abstract

1 juni 2011, Item does not contain fulltext, The mismatch repair protein, MSH3, together with MSH2, forms the MutSbeta heterodimer which recognizes and repairs base pair mismatches and larger insertion/deletion loops in DNA. Lack of specific antibodies against mouse MSH3 has hampered studies of its expression and localization. Mouse MSH3 is not immunogenic in normal mice. This problem was overcome by immunizing msh3-knockout mice and generating a panel of ten monoclonal antibodies, two of which localize MSH3 specifically in cultured mouse cells and bind to an epitope containing amino-acids 33-37. The panel also includes two antibodies that recognise both mouse and human MSH3 and bind to a conserved epitope containing amino-acids 187-194. The mouse MSH3-specific antibodies show that MSH3 is a nuclear protein with a finely-granular nucleoplasmic distribution, largely absent from areas of condensed heterochromatin. Specificity of the localization was demonstrated by absence of immunostaining in a cell line from the msh3-knockout mouse. Furthermore, we show for the first time that stress treatment of mouse cells with ethanol or hydrogen peroxide caused the re-distribution of MSH3 into nuclear bodies containing the proliferating cell nuclear antigen (PCNA), a known binding partner of MutSbeta.

Published
2011

5. The mouse mismatch repair protein, MSH3, is a nucleoplasmic protein that aggregates into denser nuclear bodies under conditions of stress.

Author

Holt, I.J., Thanh Lam, L., Tome, S., Wansink, D.G., Riele, H. te, Gourdon, G., Morris, G.E., Holt, I.J., Thanh Lam, L., Tome, S., Wansink, D.G., Riele, H. te, Gourdon, G., and Morris, G.E.

Abstract

01 juni 2011, Item does not contain fulltext, The mismatch repair protein, MSH3, together with MSH2, forms the MutSbeta heterodimer which recognizes and repairs base pair mismatches and larger insertion/deletion loops in DNA. Lack of specific antibodies against mouse MSH3 has hampered studies of its expression and localization. Mouse MSH3 is not immunogenic in normal mice. This problem was overcome by immunizing msh3-knockout mice and generating a panel of ten monoclonal antibodies, two of which localize MSH3 specifically in cultured mouse cells and bind to an epitope containing amino-acids 33-37. The panel also includes two antibodies that recognise both mouse and human MSH3 and bind to a conserved epitope containing amino-acids 187-194. The mouse MSH3-specific antibodies show that MSH3 is a nuclear protein with a finely-granular nucleoplasmic distribution, largely absent from areas of condensed heterochromatin. Specificity of the localization was demonstrated by absence of immunostaining in a cell line from the msh3-knockout mouse. Furthermore, we show for the first time that stress treatment of mouse cells with ethanol or hydrogen peroxide caused the re-distribution of MSH3 into nuclear bodies containing the proliferating cell nuclear antigen (PCNA), a known binding partner of MutSbeta.

Published
2011

6. The mouse mismatch repair protein, MSH3, is a nucleoplasmic protein that aggregates into denser nuclear bodies under conditions of stress.

Author

Holt, I.J., Thanh Lam, L., Tome, S., Wansink, D.G., Riele, H. te, Gourdon, G., Morris, G.E., Holt, I.J., Thanh Lam, L., Tome, S., Wansink, D.G., Riele, H. te, Gourdon, G., and Morris, G.E.

Abstract

01 juni 2011, Item does not contain fulltext, The mismatch repair protein, MSH3, together with MSH2, forms the MutSbeta heterodimer which recognizes and repairs base pair mismatches and larger insertion/deletion loops in DNA. Lack of specific antibodies against mouse MSH3 has hampered studies of its expression and localization. Mouse MSH3 is not immunogenic in normal mice. This problem was overcome by immunizing msh3-knockout mice and generating a panel of ten monoclonal antibodies, two of which localize MSH3 specifically in cultured mouse cells and bind to an epitope containing amino-acids 33-37. The panel also includes two antibodies that recognise both mouse and human MSH3 and bind to a conserved epitope containing amino-acids 187-194. The mouse MSH3-specific antibodies show that MSH3 is a nuclear protein with a finely-granular nucleoplasmic distribution, largely absent from areas of condensed heterochromatin. Specificity of the localization was demonstrated by absence of immunostaining in a cell line from the msh3-knockout mouse. Furthermore, we show for the first time that stress treatment of mouse cells with ethanol or hydrogen peroxide caused the re-distribution of MSH3 into nuclear bodies containing the proliferating cell nuclear antigen (PCNA), a known binding partner of MutSbeta.

Published
2011

7. Installer un chauffage ou un chauffe-eau solaire

Author

Amet, Pierre, Gourdon, G., Guern, Y., Amet, Pierre, Amet, Pierre, Gourdon, G., Guern, Y., and Amet, Pierre

Abstract

De l'installation d'un simple chauffe-eau solaire au système de chauffage avec eau chaude sanitaire, cet ouvrage pose les fondamentaux pour comprendre et s'approprier les connaissances techniques nécessaires à la réalisation d'une installation solaire thermique.La partie théorie vous permettra de concevoir correctement votre installation (dimensionnement, orientation des capteurs, transport et stockage des calories, régulation, etc.), de choisir le matériel à utiliser, et d'évaluer le montant de l'économie rendue possible par le solaire.La partie technique présente les réalisations et les témoignages de 5 constructeurs. Les choix techniques et la mise en oeuvre de chaque chantier sont décrits dans le détail, de même que leur rentabilité financière et les améliorations pouvant encore leur être apportées. Les niveaux de difficultés de ces réalisations diffèrent en fonction des compétences des autoconstructeurs : d'une simple installation solaire en direct dans le plancher chauffant gérée par une petite régulation solaire du commerce à l'utilisation d'un stockage intermédiaire dans un ballon d'eau morte associée à une régulation par automate programmable du commerce, ou même directement par ordinateur.Ce livre s'adresse aux particuliers qui souhaitent se lancer dans l'auto-installation ou simplement maîtriser le vocabulaire technique et comprendre les devis d'installateurs, ainsi qu'aux artisans et installateurs voulant compléter leurs connaissances techniques et découvrir des exemples de réalisations.

Published
2010

8. Installer un chauffage ou un chauffe-eau solaire

Author

Amet, Pierre, Gourdon, G., Guern, Y., Amet, Pierre, Amet, Pierre, Gourdon, G., Guern, Y., and Amet, Pierre

Abstract

De l'installation d'un simple chauffe-eau solaire au système de chauffage avec eau chaude sanitaire, cet ouvrage pose les fondamentaux pour comprendre et s'approprier les connaissances techniques nécessaires à la réalisation d'une installation solaire thermique.La partie théorie vous permettra de concevoir correctement votre installation (dimensionnement, orientation des capteurs, transport et stockage des calories, régulation, etc.), de choisir le matériel à utiliser, et d'évaluer le montant de l'économie rendue possible par le solaire.La partie technique présente les réalisations et les témoignages de 5 constructeurs. Les choix techniques et la mise en oeuvre de chaque chantier sont décrits dans le détail, de même que leur rentabilité financière et les améliorations pouvant encore leur être apportées. Les niveaux de difficultés de ces réalisations diffèrent en fonction des compétences des autoconstructeurs : d'une simple installation solaire en direct dans le plancher chauffant gérée par une petite régulation solaire du commerce à l'utilisation d'un stockage intermédiaire dans un ballon d'eau morte associée à une régulation par automate programmable du commerce, ou même directement par ordinateur.Ce livre s'adresse aux particuliers qui souhaitent se lancer dans l'auto-installation ou simplement maîtriser le vocabulaire technique et comprendre les devis d'installateurs, ainsi qu'aux artisans et installateurs voulant compléter leurs connaissances techniques et découvrir des exemples de réalisations.

Published
2010

9. Triplet-repeat oligonucleotide-mediated reversal of RNA toxicity in myotonic dystrophy.

Author

Mulders, S.A.M., Broek, W.J.A.A. van den, Wheeler, T.M., Croes, H.J.E., Kuik-Romeijn, P. van, Kimpe, S.J. de, Furling, D., Platenburg, G.J., Gourdon, G., Thornton, C.A., Wieringa, B., Wansink, D.G., Mulders, S.A.M., Broek, W.J.A.A. van den, Wheeler, T.M., Croes, H.J.E., Kuik-Romeijn, P. van, Kimpe, S.J. de, Furling, D., Platenburg, G.J., Gourdon, G., Thornton, C.A., Wieringa, B., and Wansink, D.G.

Abstract

Contains fulltext : 81075.pdf (publisher's version ) (Closed access), Myotonic dystrophy type 1 (DM1) is caused by toxicity of an expanded, noncoding (CUG)n tract in DM protein kinase (DMPK) transcripts. According to current evidence the long (CUG)n segment is involved in entrapment of muscleblind (Mbnl) proteins in ribonuclear aggregates and stabilized expression of CUG binding protein 1 (CUGBP1), causing aberrant premRNA splicing and associated pathogenesis in DM1 patients. Here, we report on the use of antisense oligonucleotides (AONs) in a therapeutic strategy for reversal of RNA-gain-of-function toxicity. Using a previously undescribed mouse DM1 myoblast-myotube cell model and DM1 patient cells as screening tools, we have identified a fully 2'-O-methyl-phosphorothioate-modified (CAG)7 AON that silences mutant DMPK RNA expression and reduces the number of ribonuclear aggregates in a selective and (CUG)n-length-dependent manner. Direct administration of this AON in muscle of DM1 mouse models in vivo caused a significant reduction in the level of toxic (CUG)n RNA and a normalizing effect on aberrant premRNA splicing. Our data demonstrate proof of principle for therapeutic use of simple sequence AONs in DM1 and potentially other unstable microsatellite diseases.

Published
2009

10. Triplet-repeat oligonucleotide-mediated reversal of RNA toxicity in myotonic dystrophy.

Author

Mulders, S.A.M., Broek, W.J.A.A. van den, Wheeler, T.M., Croes, H.J.E., Kuik-Romeijn, P. van, Kimpe, S.J. de, Furling, D., Platenburg, G.J., Gourdon, G., Thornton, C.A., Wieringa, B., Wansink, D.G., Mulders, S.A.M., Broek, W.J.A.A. van den, Wheeler, T.M., Croes, H.J.E., Kuik-Romeijn, P. van, Kimpe, S.J. de, Furling, D., Platenburg, G.J., Gourdon, G., Thornton, C.A., Wieringa, B., and Wansink, D.G.

Abstract

Contains fulltext : 81075.pdf (publisher's version ) (Closed access), Myotonic dystrophy type 1 (DM1) is caused by toxicity of an expanded, noncoding (CUG)n tract in DM protein kinase (DMPK) transcripts. According to current evidence the long (CUG)n segment is involved in entrapment of muscleblind (Mbnl) proteins in ribonuclear aggregates and stabilized expression of CUG binding protein 1 (CUGBP1), causing aberrant premRNA splicing and associated pathogenesis in DM1 patients. Here, we report on the use of antisense oligonucleotides (AONs) in a therapeutic strategy for reversal of RNA-gain-of-function toxicity. Using a previously undescribed mouse DM1 myoblast-myotube cell model and DM1 patient cells as screening tools, we have identified a fully 2'-O-methyl-phosphorothioate-modified (CAG)7 AON that silences mutant DMPK RNA expression and reduces the number of ribonuclear aggregates in a selective and (CUG)n-length-dependent manner. Direct administration of this AON in muscle of DM1 mouse models in vivo caused a significant reduction in the level of toxic (CUG)n RNA and a normalizing effect on aberrant premRNA splicing. Our data demonstrate proof of principle for therapeutic use of simple sequence AONs in DM1 and potentially other unstable microsatellite diseases.

Published
2009

11. Triplet-repeat oligonucleotide-mediated reversal of RNA toxicity in myotonic dystrophy.

Author

Mulders, S.A.M., Broek, W.J.A.A. van den, Wheeler, T.M., Croes, H.J.E., Kuik-Romeijn, P. van, Kimpe, S.J. de, Furling, D., Platenburg, G.J., Gourdon, G., Thornton, C.A., Wieringa, B., Wansink, D.G., Mulders, S.A.M., Broek, W.J.A.A. van den, Wheeler, T.M., Croes, H.J.E., Kuik-Romeijn, P. van, Kimpe, S.J. de, Furling, D., Platenburg, G.J., Gourdon, G., Thornton, C.A., Wieringa, B., and Wansink, D.G.

Abstract

Contains fulltext : 81075.pdf (publisher's version ) (Closed access), Myotonic dystrophy type 1 (DM1) is caused by toxicity of an expanded, noncoding (CUG)n tract in DM protein kinase (DMPK) transcripts. According to current evidence the long (CUG)n segment is involved in entrapment of muscleblind (Mbnl) proteins in ribonuclear aggregates and stabilized expression of CUG binding protein 1 (CUGBP1), causing aberrant premRNA splicing and associated pathogenesis in DM1 patients. Here, we report on the use of antisense oligonucleotides (AONs) in a therapeutic strategy for reversal of RNA-gain-of-function toxicity. Using a previously undescribed mouse DM1 myoblast-myotube cell model and DM1 patient cells as screening tools, we have identified a fully 2'-O-methyl-phosphorothioate-modified (CAG)7 AON that silences mutant DMPK RNA expression and reduces the number of ribonuclear aggregates in a selective and (CUG)n-length-dependent manner. Direct administration of this AON in muscle of DM1 mouse models in vivo caused a significant reduction in the level of toxic (CUG)n RNA and a normalizing effect on aberrant premRNA splicing. Our data demonstrate proof of principle for therapeutic use of simple sequence AONs in DM1 and potentially other unstable microsatellite diseases.

Published
2009

12. Barclayrama / Eddie Barclay et son grand orchestre

Author

Wayne, Johnny (1918-1990). Auteur ou responsable intellectuel, Wal-Berg (1910-1994). Auteur ou responsable intellectuel, Stern, Emil (1913-1997). Auteur ou responsable intellectuel, Richin, André. Auteur ou responsable intellectuel, Pozzaglio, R.. Auteur ou responsable intellectuel, Perret, Pierre (1934-....). Auteur ou responsable intellectuel, Monnot, Marguerite (1903-1961). Auteur ou responsable intellectuel, Modugno, Domenico (1928-1994). Auteur ou responsable intellectuel, Marnay, Eddy (1920-2003). Auteur ou responsable intellectuel, Lemarque, Francis (1917-2002). Auteur ou responsable intellectuel, Lafforgue, René-Louis (1928-1967). Auteur ou responsable intellectuel, Lacome, Jacques. Auteur ou responsable intellectuel, Gourdon, G.. Auteur ou responsable intellectuel, Gimby, Bobby (1918-1998). Auteur ou responsable intellectuel, Delanoë, Pierre (1918-2006). Auteur du texte, Corazza, Rémy (19..-.... ; compositeur). Auteur ou responsable intellectuel, Cantoral Garcia, Roberto (1935-2010). Auteur ou responsable intellectuel, Calfati, Bob. Auteur ou responsable intellectuel, Bonifay, Fernand (1920-1993). Auteur ou responsable intellectuel, Barclay, Eddie (1921-2005). Direction d'orchestre, Wayne, Johnny (1918-1990). Auteur ou responsable intellectuel, Wal-Berg (1910-1994). Auteur ou responsable intellectuel, Stern, Emil (1913-1997). Auteur ou responsable intellectuel, Richin, André. Auteur ou responsable intellectuel, Pozzaglio, R.. Auteur ou responsable intellectuel, Perret, Pierre (1934-....). Auteur ou responsable intellectuel, Monnot, Marguerite (1903-1961). Auteur ou responsable intellectuel, Modugno, Domenico (1928-1994). Auteur ou responsable intellectuel, Marnay, Eddy (1920-2003). Auteur ou responsable intellectuel, Lemarque, Francis (1917-2002). Auteur ou responsable intellectuel, Lafforgue, René-Louis (1928-1967). Auteur ou responsable intellectuel, Lacome, Jacques. Auteur ou responsable intellectuel, Gourdon, G.. Auteur ou responsable intellectuel, Gimby, Bobby (1918-1998). Auteur ou responsable intellectuel, Delanoë, Pierre (1918-2006). Auteur du texte, Corazza, Rémy (19..-.... ; compositeur). Auteur ou responsable intellectuel, Cantoral Garcia, Roberto (1935-2010). Auteur ou responsable intellectuel, Calfati, Bob. Auteur ou responsable intellectuel, Bonifay, Fernand (1920-1993). Auteur ou responsable intellectuel, and Barclay, Eddie (1921-2005). Direction d'orchestre

Abstract

Titre uniforme : [Irma la douce]. Extrait, Titre uniforme : [Lazzarelle], Titre uniforme : [Marjolaine], Titre uniforme : [Julie la rousse], Comprend : Marjolaine : slow-fox / F. Lemarque - Julie la rousse : valse / R. L. Lafforgue - TESORO MIO : fox / A. Richin ; B. Calfati - La Vallée des larmes : boléro / E. Marnay ; E. Stern ; Walberg - Tango des Pyrénées / F. Bonifay ; G. Gourdon ; F. Antonini - Moi, j'attends Adèle : fox / P. Perret ; R. Corazza - Avec les anges : valse / M. Monnot - Lazzarelle : fox / D. Modugno ; R. Pozzaglio - Tu n'as pas très bon caractère : fox / Albaux - Histoire d'un amour : boléro / Almaran - Si je t'envoie des fraises : fox / P. Perret ; R. Corazza - La Môme aux boutons : valse / Lacome - Le Poney aux étoiles : slow-fox / E. Marnay ; E. Stern - THE CRICKETT-SONG : fox / B. Gimby ; J. Wayne - Pardon : boléro / P. Delanoë ; R. Contaral - Les Marches de Provence : fox / G. Bécaud, BnF-Partenariats, Collection sonore - Believe, Contient une table des matières

Published
1959

13. Correspondance de Gaston Paris. I-XXXIV Lettres adressées à Gaston Paris. XII Gevaert-Guiffrey.

Author

Paris, Gaston (1839-1903). Destinataire de lettres, Gevaert, François Auguste. Auteur de lettres, Giannini, Giovanni. Auteur de lettres, Gilbert, O. Auteur de lettres, Gillet, Horace. Auteur de lettres, Gillet, Louis. Auteur de lettres, Gilliéron, J. Auteur de lettres, Gillot, H. Auteur de lettres, Girard, Jules. Auteur de lettres, Girard, Julien. Auteur de lettres, Giraud, Victor. Auteur de lettres, Girelet, Ch. Auteur de lettres, Giry, Arthur. Auteur de lettres, Glaser, Emmanuel. Auteur de lettres, Glaser, Victor. Auteur de lettres, Glehm, A.V. Auteur de lettres, Gletron (Mme). Auteur de lettres, Gloth, W. Auteur de lettres, Godefroy, Frédéric. Auteur de lettres, Godefroy, Valentine. Auteur de lettres, Goelzer, Henri. Auteur de lettres, Goetz, Alphonse. Auteur de lettres, Goldbeck, Carl. Auteur de lettres, Goldschmidt, M. Auteur de lettres, Gollancz, J. Auteur de lettres, Golther, W. Auteur de lettres, Gonzalvez-Vianna. Auteur de lettres, Gordon, A.D. Auteur de lettres, Gorra, E. Auteur de lettres, Goresio, Gaspare. Auteur de lettres, Gossard, Ernest. Auteur de lettres, Gourdon, G. Auteur de lettres, Gourjault, H. de. Auteur de lettres, Gourjault, Olivier de. Auteur de lettres, Gourmont, Rémy de. Auteur de lettres, Graaff, E.C. de. Auteur de lettres, Graevell. Auteur de lettres, Graf, Arturo. Auteur de lettres, Graffin, Monseigneur René. Auteur de lettres, Grammont, Maurice. Auteur de lettres, Grand, Ernest-Daniel. Auteur de lettres, Grand, Mme Mary. Auteur de lettres, Grandgagnage, Maurice. Auteur de lettres, Grandgent, C.H. Auteur de lettres, Gras, Louis-Pierre. Auteur de lettres, Graux, Charles. Auteur de lettres, Gréard, Octave. Auteur de lettres, Gregorio, Giacomo de. Auteur de lettres, Grelé, Eugène. Auteur de lettres, Grenier, Édouard. Auteur de lettres, Grienberger, Theodor von. Auteur de lettres, Gröber, Gustav. Auteur de lettres, Grosjean, Oscar. Auteur de lettres, Groth, Ernst. Auteur de lettres, Gruener, Gustav. Auteur de lettres, Grunwald, Maurice. Auteur de lettres, Guardia, J.M. Auteur de lettres, Guarnerio, Ph. Auteur de lettres, Guastella, Serafmo Amabile. Auteur de lettres, Gubernatis, Angelo de. Auteur de lettres, Guennou, Ch. Auteur de lettres, Guerlin de Guer, Ch. Auteur de lettres, Guerne, André-Charles-Romain. Auteur de lettres, Guerrini, Olindo. Auteur de lettres, Guesnon, M. Auteur de lettres, Guessard, Francis. Auteur de lettres, Guibal, G. Auteur de lettres, Guichot, Alexandre. Auteur de lettres, Guieysse, Paul. Auteur de lettres, Guiffrey, Jules-Joseph. Auteur de lettres, Paris, Gaston (1839-1903). Destinataire de lettres, Gevaert, François Auguste. Auteur de lettres, Giannini, Giovanni. Auteur de lettres, Gilbert, O. Auteur de lettres, Gillet, Horace. Auteur de lettres, Gillet, Louis. Auteur de lettres, Gilliéron, J. Auteur de lettres, Gillot, H. Auteur de lettres, Girard, Jules. Auteur de lettres, Girard, Julien. Auteur de lettres, Giraud, Victor. Auteur de lettres, Girelet, Ch. Auteur de lettres, Giry, Arthur. Auteur de lettres, Glaser, Emmanuel. Auteur de lettres, Glaser, Victor. Auteur de lettres, Glehm, A.V. Auteur de lettres, Gletron (Mme). Auteur de lettres, Gloth, W. Auteur de lettres, Godefroy, Frédéric. Auteur de lettres, Godefroy, Valentine. Auteur de lettres, Goelzer, Henri. Auteur de lettres, Goetz, Alphonse. Auteur de lettres, Goldbeck, Carl. Auteur de lettres, Goldschmidt, M. Auteur de lettres, Gollancz, J. Auteur de lettres, Golther, W. Auteur de lettres, Gonzalvez-Vianna. Auteur de lettres, Gordon, A.D. Auteur de lettres, Gorra, E. Auteur de lettres, Goresio, Gaspare. Auteur de lettres, Gossard, Ernest. Auteur de lettres, Gourdon, G. Auteur de lettres, Gourjault, H. de. Auteur de lettres, Gourjault, Olivier de. Auteur de lettres, Gourmont, Rémy de. Auteur de lettres, Graaff, E.C. de. Auteur de lettres, Graevell. Auteur de lettres, Graf, Arturo. Auteur de lettres, Graffin, Monseigneur René. Auteur de lettres, Grammont, Maurice. Auteur de lettres, Grand, Ernest-Daniel. Auteur de lettres, Grand, Mme Mary. Auteur de lettres, Grandgagnage, Maurice. Auteur de lettres, Grandgent, C.H. Auteur de lettres, Gras, Louis-Pierre. Auteur de lettres, Graux, Charles. Auteur de lettres, Gréard, Octave. Auteur de lettres, Gregorio, Giacomo de. Auteur de lettres, Grelé, Eugène. Auteur de lettres, Grenier, Édouard. Auteur de lettres, Grienberger, Theodor von. Auteur de lettres, Gröber, Gustav. Auteur de lettres, Grosjean, Oscar. Auteur de lettres, Groth, Ernst. Auteur de lettres, Gruener, Gustav. Auteur de lettres, Grunwald, Maurice. Auteur de lettres, Guardia, J.M. Auteur de lettres, Guarnerio, Ph. Auteur de lettres, Guastella, Serafmo Amabile. Auteur de lettres, Gubernatis, Angelo de. Auteur de lettres, Guennou, Ch. Auteur de lettres, Guerlin de Guer, Ch. Auteur de lettres, Guerne, André-Charles-Romain. Auteur de lettres, Guerrini, Olindo. Auteur de lettres, Guesnon, M. Auteur de lettres, Guessard, Francis. Auteur de lettres, Guibal, G. Auteur de lettres, Guichot, Alexandre. Auteur de lettres, Guieysse, Paul. Auteur de lettres, and Guiffrey, Jules-Joseph. Auteur de lettres

Abstract

Contient : Gevaert, François Auguste, directeur du Conservatoire de Bruxelles. Lettre(s) ; Giannini, Giovanni. Lettre(s) ; Gilbert, O.. Lettre(s) ; Gillet, Horace. Lettre(s) ; Gillet, Louis, de l'Académie française. Lettre(s) ; Gilliéron, J.. Lettre(s) ; Gillot, H., professeur. Lettre(s) ; Girard, Jules, membre de l'Institut. Lettre(s) ; Girard, Julien, proviseur de lycée. Lettre(s) ; Giraud, Victor, critique littéraire. Lettre(s) ; Girelet, Ch.. Lettre(s) ; Giry, Arthur, professeur à l'École des Chartes, membre de l'Institut. Lettre(s) ; Glaser, Emmanuel. Lettre(s) ; Glaser, Victor. Lettre(s) ; Glehm, A.V.. Lettre(s) ; Gletron (Mme). Lettre(s) ; Gloth, W.. Lettre(s) ; Godefroy, Frédéric, philologue. Lettre(s) ; Godefroy, Valentine, Mme Frédéric. Lettre(s) ; Goelzer, Henri, professeur à la Sorbonne. Lettre(s) ; Goetz, Alphonse. Lettre(s) ; Goldbeck, Carl. Lettre(s) ; Goldschmidt, M.. Lettre(s) ; Gollancz, J., professeur à Cambridge. Lettre(s) ; Golther, W., professeur à l'Université de Munich. Lettre(s) ; Gonzalvez-Vianna. Lettre(s) ; Gordon, A.D.. Lettre(s) ; Gorra, E.. Lettre(s) ; Goresio, Gaspare. Lettre(s) ; Gossard, Ernest, conservateur de la Bibliothèque royale de Bruxelles. Lettre(s) ; Gourdon, G., journaliste. Lettre(s) ; Gourjault, H. de. Lettre(s) ; Gourjault, Olivier de. Lettre(s) ; Gourmont, Rémy de, écrivain. Lettre(s) ; Graaff, E.C. de. Lettre(s) ; Graevell. Lettre(s) ; Graf, Arturo, recteur de l'Université de Turin. Lettre(s) ; Graffin, Monseigneur René. Lettre(s) ; Grammont, Maurice, professeur à l'Université de Montpellier. Lettre(s) ; Grand, Ernest-Daniel, archiviste-paléographe. Lettre(s) ; Grand, Mme Mary. Lettre(s) ; Grandgagnage, Maurice. Lettre(s) ; Grandgent, C.H., professeur à Harvard. Lettre(s) ; Gras, Louis-Pierre, bibliothécaire à Montbrison. Lettre(s) ; Graux, Charles, bibliothécaire à la Sorbonne. Lettre(s) ; Gréard, Octave, recteur de l'Université de Paris. Lettre(s) ; Gregorio, Giacomo de. Lettre(s) ; Grelé, Eugène, journaliste. Lettr, Numérisation effectuée à partir d'un document original : NAF 24441.

14. The expansion of 300 CTG repeats in myotonic dystrophy transgenic mice does not induce sensory or motor neuropathy

Author

Gantelet, E., Kraftsik, R., Delaloye, S., Gourdon, G., Kuntzer, T., Barakat-Walter, I., Gantelet, E., Kraftsik, R., Delaloye, S., Gourdon, G., Kuntzer, T., and Barakat-Walter, I.

Abstract

Summary: Although many studies have been carried out to verify the involvement of the peripheral nervous system (PNS) in dystrophia myotonica (DM1) patients, the results remain controversial. The generation of DM1 transgenic mice displaying the human DM1 phenotype provides a useful tool to investigate the type and incidence of structural abnormalities in the PNS. In the present study, the morphological and morphometric analysis of semi-thin sections of sciatic and sural nerves, lumbar dorsal root ganglia (DRG) and lumbar spinal cords revealed that in DM1 transgenic mice carrying 300 CTG repeats, there is no change in the number and diameter of myelinated axons compared to wild type. Only a non-significant reduction in the percentage of thin myelinated axons was detected in electron micrographs of ultra-thin sciatic nerve sections. Analysis of the number of neurons did not reveal a loss in number of either sensory neurons in the lumbar DRG or motor neurons in the lumbar spinal cord in these DM1 mice. Furthermore, in hind limb muscle sections, stained with a neurofilament antibody and α-bungarotoxin, the intramuscular axon arborization appeared normal in DM1 mice and undistinguishable from that in wild-type mice. Moreover, in DM1 mice, there was no irregularity in the structure or an increase in the endplate area. Also statistical analysis did not show an increase in endplate density or in the concentration of acetylcholine receptors. Altogether, these results suggest that 300 CTG repeats are not sufficient to induce axonopathy, demyelination or neuronopathies in this transgenic mouse model

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