top of page

FRIBOURG LAB

Ancre 1

PUBLICATIONS

2023
53. Thore S., Raoelijaona F., Talenton V., Fribourg S, Mackereth C.D. (2023) Molecular details of the CPSF73-CPSF100 C-terminal heterodimer and interaction with Symplekin. Open Biology, 13:230221. doi:10.1098/rsob.230221.

52. Cao M., Vial A., Minder L., Guédin A., Fribourg S., Azéma L., Feuillie C., Molinari M., Di Primo C., Barthélémy P., Leblond Chain J. (2023) Aptamer-based nanotrains and nanoflowers as quinine delivery systems. Int J Pharm X. doi: 10.1016/j.ijpx.2023.100172.

51.1H, 15N, 13C resonance assignments of a minimal CPSF73-CPSF100 C-terminal heterodimer Biomol NMR assignments (2023) Thore S., Fribourg S., Mackereth C.D. Biomol NMR Assign. doi: 10.1007/s12104-023-10118-6.

2022
50. Schell B., Legrand P., Fribourg S. Crystal structure of SFPQ-NONO heterodimer. (2022) Biochimie. Mar 1;198:1-7. doi: 10.1016/j.biochi.2022.02.011.

49. Djordjevic D, Pinard M, Gauthier MS, Smith-Hicks C, Hoffman TL, Wolf NI, Oegema R, van Binsbergen E, Baskin B, Bernard G, Fribourg S, Coulombe B, Yoon G De novo variants in POLR3B cause ataxia, spasticity, and demyelinating neuropathy. Am J Hum Genet. 2022 Apr 7;109(4):759-763. doi: 10.1016/j.ajhg.2022.03.006.

2021
48. De novo variants in POLR3B cause ataxia, spacticity and demyelinating neuropathy. D. Djordjevic, M. Pinard, M.S. Gauthier, C. Smith-Hicks, T. L. Hoffman, N.I. Wolf, R. Oegema, E. van Binsbergen, B. Baskin, G. Bernard, S. Fribourg, B. Coulombe, G. Yoon (2021) Am. J. Hum. Genet., 108, 186-193. doi: 10.1016/j.ajhg.2020.12.002

2020

47. Guichard G, Cussol L, Mauran L, Buratto J, Belorusova AY, Neuville M, Osz J, Fribourg S, Fremaux J, Dolain C, Goudreau S, Rochel N. Structural basis for α-helix mimicry and inhibition of protein-protein interactions with oligourea foldamers. Angew Chem Int Ed Engl. (2020) doi: 10.1002/anie.202008992

2019

46. L. Gauquelin et al. Clinical Spectrum of POLR3-Related Leukodystrophy caused by biallelic POLR1C pathogenic variants (2019) Neurol Genet. Oct 30;5(6):e369. doi: 10.1212/NXG.0000000000000369.

45. F. Maurice, N. Pérébaskine, S. Thore, S. Fribourg In vitro dimerization of hRIO2 (2019) RNA Biology, 16, 1633-1642. doi: 10.1080/15476286.2019.1653679

44. L. El Ayoubi, H. Dumay-Odelot, A. Chernev, F. Boissier, L. Minvielle-Sébastia, H. Urlaub, S. Fribourg, M. Teichmann The hRPC62 subunit of human RNA polymerase III displays helicase activity (2019) Nucleic Acids Res., Nov 4;47(19):10313-10326. . doi: 10.1093/nar/gkz788

43. S. Thore & S. Fribourg. Structural insigths into the 3'-end mRNA maturation machinery: snapshot on polyadenylation signal recognition (2019) Biochimie, doi:10.1016/j.biochi.2019.03.016

2018

42. F. Raoelijaona, S. Thore*, S. Fribourg* Domain definition and interaction mapping for the endonuclease complex hNob1/hPno1 (2018) RNA Biology, 15(9), 1174-1180, doi: 10.1080/15476286.2018.1517013

41. N. Pérébaskine, S. Thore, S. Fribourg* Structural and interaction of Rrp5 C-terminal region (2018) FEBS Open Bio, 8(10), 1605-1614. doi: 10.1002/2211-5463.12495.

40. C. Joret et al. The Npa1p complex chaperones the assembly of the earliest eukaryotic large ribosomal precursor (2018) PLoS Genet. 2018 Aug 31;14(8):e1007597. doi: 10.1371/journal.pgen.1007597

39. M.E. Carter-Timofte et al. Varicella-zoster virus CNS vasculitis and RNA polymerase III defect in identical twins (2018) Neurol Neuroimmunol Neuroinflamm. 2018 Sep 7;5(6):e500. doi: 10.1212/NXI.0000000000000500.

2017

38. J. Guéguéniat*, A.F. Dupin*, J. Stojko, L. Beaurepaire, S. Sanglier-Cianferani, C.D. Mackereth, L. Minvielle-Sébastia, S. Fribourg Distinct roles of Pcf11 zinc-binding domains in pre-mRNA 3'-end processing (2017) Nucleic Acids Res. 45(17), 10115-10131. doi:10.1093/nar/gkx674.

37. Ogunjimi et al. Inborn errors in RNA polymerase III underlie severe varicella zoster virus infections (2017) J. Clin. Invest.,127(9), 3543-3556. doi:10.1172/JCI92280.

36. F. Boissier*, C.M. Schmidt*, J. Linnemann, S. Fribourg*, J. Perez-Fernandez*. Pwp2 mediates UTP-B assembly via two structurally independent domains (2017) Sci. Rep., 7(1):3169. doi: 10.1038/s41598-017-03034-y

35. J. Stojko*, A. Dupin*, S. Chaignepain*, L. Beaurepaire, A. Vallet-Courbin, A. Van Dorsselaer, J.M. Schmitter, L. Minvielle-Sebastia, S. Fribourg*, S. Cianferani*. Structural characterization of the yeast CF IA complex through a combination of mass spectrometry approaches (2017), IJMS, 420, 57-66. doi.org/10.1016/j.ijms.2016.08.005

2016

34. F. Frénois, P. Legrand, S. Fribourg. Sqt1 is an eight-bladed WD40 protein (2016) Acta Cryst. F., 72, 59-64.

2015

33. X. Xu, N. Pérébaskine, Minvielle-Sebastia L., S. Fribourg, C.D. Mackereth. Chemical shift assignments of a new folded domain from yeast Pcf11 (2015) Biomol. NMR assign, 9(2):421-425.

32. Thiffault et al. Recessive mutations in POLR1C cause a leukodystrophy by impairing biogenesis of RNA polymerase III (2015) Nat. Comm, 6, 7623.

31. F. Boissier, H. Dumay-Odelot, M. Teichmann, S. Fribourg. Structural analysis of RPC32b - RPC62 complex (2015) J. Struct. Biol, 192, 313-319.

2014

30. A. Delprato, Y. Al Kadri, N. Pérébaskine, C. Monfoulet, Y. Henry, A.K. Henras, S. Fribourg. Crucial role of the Rcl1p-Bms1p interaction for yeast pre-ribosomal RNA processing (2014) Nucleic Acids Res., 45(15), 10161-72.

29. N. Wolf, A. Vanderver, R. van Spaendonk, R. Schiffmann, B. Brais, M. Bugiani, E. Sistermans, C. Catsman-Berrevoets, J. Kros, P. Soares Pinto, D. Pohl, S. Tirupathi, P. Stromme, T. de Grauw, S. Fribourg, M. Demos, A. Pizzino, S. Naidu, K. Guerrero, M. S. van der Knaap, and G. Bernard. Clinical spectrum of 4H leukodystrophy caused by POLR3A and POLR3B mutations (2014) Neurology, 18, 1898-1905.

28. Dupin A. & Fribourg S. (2014). Structural basis for ATP loss by Clp1p in a G135R mutant protein. Biochimie, 101, 203-207.

2012

27. Haddad R., Maurice F., Viphakone N., Voisinet-Hakil F., Fribourg S, Minvielle-Sebastia L. (2012). An essential role for Clp1 in assembly of polyadenylation complex CF IA and Pol II transcription termination. Nucleic Acids Res., 40, 1226-1239.

26. Teichmann M., Dumay-Odelot H., Fribourg S. (2012). Structural and functional aspects of winged-helix domains at the core of transcription initiation complexes. Transcription, 3, 2-7.

2011

25. Lefevre S, Dumay-Odelot H, El Ayoubi L, Budd A, Legrand P, Pinaud N, Teichmann M, Fribourg S (2011) Structure-function analysis of hRPC62 provides insights into RNA polymerase III transcription initiation. Nat. Struct. Mol. Biol., 18(3), 352-358.

24. Moreno-Morcillo M, Mackereth CD, Minvielle-Sébastia L, Fribourg S (2011) Hexameric architecture of CstF supported by CstF-50 homodimerization domain structure. RNA,17, 412-418.

23. Diebold ML, Fribourg S, Kocj M, Metzger T, Romier C (2011) Deciphering correct strategies for multiprotein complex assembly by co-expression: applications to complexes as large as the histone-octamer. J. Struct. Biol., 175, 178-188.

22. Moreno-Morcillo M, Minvielle-Sebastia L, Fribourg S, Mackereth CD (2011) Locked tether formation by cooperative folding of Rna14p monkeytail and Rna15p hinge domains in the yeast CF IA complex. Structure, 19, 534-545.

21. Bouchecareilh M, Higa A, Fribourg S, Moenner M, Chevet E (2011) Peptides derived from the bifunctional kinase/RNase enzyme IRE1a modulate IRE1a activity and protect cells from endoplasmic reticulum stress. FASEB J, 25, 3115-3129.

20. Bernard G, Chouey E, Putorti ML, Tetreault T, Takanohashi A, Carasso G, Clement I, Boespflug-Tanguy O, Fribourg S, Teichmann M, Megarbane A, Schiffmann R, Vanderver A, Brais B(2011) Mutation of the RNA polymerase III subunit POLR3A cause a recessive hypomyelinating leukodystrophy. Am. J. Hum. Genet., 89, 415-423.

19. Tetreault M., Choquet K., Orcesi S., Tonduti D., Balotin U., Teichmann M., Fribourg S, Schiffmann R., Brais B., Vanderver A., Bernard G. (2011). Recessive mutations in POLR3B, encoding the second largest subunit of Pol III, cause hypomyelinating leukodystrohpy. Am. J. Hum. Genet., 89, 652-655.

2009

18. Aguissa-Touré AH, Da Costa L, Leblanc T, Tchernia G, Fribourg S, Gleizes PE (2009) Diamond-Blackfan anemia reveals the dark side of ribosome biogenesis. Med Sci., 25, 69-76.

2008

17. Choesmel V, Fribourg S, Aguissa-Touré AH, Pinaud N, Legrand P, Gazda HT & Gleizes PE. (2008) Mutation of ribosomal protein RPS24 in Diamond-Blackfan anemia results in a ribosome biogenesis disorder. Hum Mol Genet.,17, 1253-1263.

16. Lebars I, Legrand P, Aimé A, Pinaud N, Fribourg S, Di Primo C. (2008) Exploring TAR-RNA aptamer loop-loop interaction by X-ray crystallography, UV spectroscopy and surface plasmon resonance. Nucleic Acids Res., 36, 7146-56.

2007

15. Choesmel V, Bacqueville D, Rouquette J, Noaillac-Depeyre J, Fribourg S, Crétien A, Leblanc T, Tchernia G, Da Costa L, Gleizes PE. (2007) Impaired ribosome biogenesis in Diamond-Blackfan anemia. Blood. 109, 1275-1283.

14. Gregory L.A., Aguissa-Touré A.H., Legrand P., Pinaud N., Gleizes P.E. & Fribourg S. (2007) Molecular basis of Diamond-Blackfan anemia: structure and function analysis of RPS19. Nucleic Acids Res., 35, 5913-5921.

13. Legrand P., Pinaud N., Minvielle-Sebastia L. & Fribourg S. (2007) The structure of CstF-77 provides insights into CstF assembly. Nucleic Acids Res., 35, 4515-4522.

2005

12. Kellenberger E, Dominguez C, Fribourg S, Wasielewski E, Moras D, Poterszman A, Boelens R, Kieffer B. (2005) Solution structure of the C-terminal domain of TFIIH P44 subunit reveals a novel type of C4C4 ring domain involved in protein-protein interactions. J Biol Chem. 27;280(21):20785-92.

11. Lorentzen E., Walter P., Fribourg S., Evguenieva-Hackenberg E., Klug G. & Conti E. (2005) The The archeal exosome core is an hexameric ring with three catalytic subunits. Nat. Struct. Mol. Biol, 12, 575-581.

2003

10. Fribourg S., Gatfield D., Izaurralde E. & Conti E (2003) A novel mode of RBD-protein recognition in the Y14-Mago complex. Nat. Struct. Biol.,10, 433-439.

9. Fribourg S. & Conti E. (2003) Structural similarity in the absence of sequence homology of the messenger RNA export factors Mtr2 and p15. EMBO Rep., 4, 699-703 .

2002

8. Jawhari A., Uhring M. Crucifix C., Fribourg S., Schultz P., Poterszman A., Egly J.M. & Moras D. (2002) Expression of FLAG fusion proteins in insect cells: application to the multi-subunit transcription/DNA repair factor TFIIH. J. Biol. Chem., 24, 513-523.

2001

7. Fribourg S., Braun I., Izaurralde E. & Conti E (2001) Structural basis for the recognition of a nucleoporin FG repeat by the NTF2-like domain of TAP/p15 mRNA nuclear export factor. Mol. Cell, 8, 645-655.

6. Fribourg S., Romier C, Werten S., Gangloff Y.G., Poterszmann A., Moras D. (2001) Dissecting the interaction network by pairwise coexpression of subunits in E. coli. J. Mol. Biol., 306, 363-373.

1995 - 2000

5. Schultz P, Fribourg S., Poterszman A., Mallouh V., Moras D. & Egly J.M. (2000) Molecular structure of human TFIIH. Cell, 102, 599-607.

4. Fribourg S., Kellenberger E., Rogniaux H., Poterszman A., Van Dorsselaer A., Thierry J.C., Egly J.M., Moras D., Kieffer B. (2000) Strucutral characterization of the cysteine-rich domain of TFIIH p44 subunit. J. Biol. Chem., 275, 31963-31971.

3. Coin F, Marinoni J.C., Rodolfo C., Fribourg S., Pedrini A.M. & Egly J.M. (1998) Mutations in the XPD gene result in XP and TTD phenotypes, preventing interaction between XPD and the p44 subunit of TFIIH. Nat. Genet., 20, 184-189 .

2. Trucco C., Flatter E., Fribourg S., de Murcia G. & Menissier-de Murcia J. (1996) Mutations in the amino-terminal domain of the human poly(ADP-ribose) polymerase that affect its catalytic activity but not its DNA binding capacity. FEBS Lett., 399, 313-316.

1. Masson M. et al. (1995) Poly(ADP-ribose) polymerase: structure-function relationship. Biochimie, 77, 456-461.

bottom of page