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Sebastien Fribourg
Sebastien Fribourg
INSERM, Université de Bordeaux, Université de Strasbourg, EMBL
Verified email at inserm.fr
Title
Cited by
Cited by
Year
Mutations in the XPD helicase gene result in XP and TTD phenotypes, preventing interaction between XPD and the p44 subunit of TFIIH
F Coin, JC Marinoni, C Rodolfo, S Fribourg, AM Pedrini, JM Egly
Nature genetics 20 (2), 184-188, 1998
4311998
Structural basis for the recognition of a nucleoporin FG repeat by the NTF2-like domain of the TAP/p15 mRNA nuclear export factor
S Fribourg, IC Braun, E Izaurralde, E Conti
Molecular cell 8 (3), 645-656, 2001
2892001
Impaired ribosome biogenesis in Diamond-Blackfan anemia
V Choesmel, D Bacqueville, J Rouquette, J Noaillac-Depeyre, S Fribourg, ...
Blood 109 (3), 1275-1283, 2007
2682007
Mutations of POLR3A encoding a catalytic subunit of RNA polymerase Pol III cause a recessive hypomyelinating leukodystrophy
G Bernard, E Chouery, ML Putorti, M Tétreault, A Takanohashi, G Carosso, ...
The American Journal of Human Genetics 89 (3), 415-423, 2011
2552011
Molecular structure of human TFIIH
P Schultz, S Fribourg, A Poterszman, V Mallouh, D Moras, JM Egly
Cell 102 (5), 599-607, 2000
2502000
The archaeal exosome core is a hexameric ring structure with three catalytic subunits
E Lorentzen, P Walter, S Fribourg, E Evguenieva-Hackenberg, G Klug, ...
Nature structural & molecular biology 12 (7), 575-581, 2005
2332005
A novel mode of RBD-protein recognition in the Y14–Mago complex
S Fribourg, D Gatfield, E Izaurralde, E Conti
Nature Structural & Molecular Biology 10 (6), 433-439, 2003
2142003
Clinical spectrum of 4H leukodystrophy caused by POLR3A and POLR3B mutations
NI Wolf, A Vanderver, RML Van Spaendonk, R Schiffmann, B Brais, ...
Neurology 83 (21), 1898-1905, 2014
1932014
Recessive mutations in POLR3B, encoding the second largest subunit of Pol III, cause a rare hypomyelinating leukodystrophy
M Tétreault, K Choquet, S Orcesi, D Tonduti, U Balottin, M Teichmann, ...
The American Journal of Human Genetics 89 (5), 652-655, 2011
1642011
Recessive mutations in POLR1C cause a leukodystrophy by impairing biogenesis of RNA polymerase III
I Thiffault, NI Wolf, D Forget, K Guerrero, LT Tran, K Choquet, ...
Nature communications 6 (1), 7623, 2015
1602015
Mutation of ribosomal protein RPS24 in Diamond-Blackfan anemia results in a ribosome biogenesis disorder
V Choesmel, S Fribourg, AH Aguissa-Touré, N Pinaud, P Legrand, ...
Human molecular genetics 17 (9), 1253-1263, 2008
1452008
Deciphering correct strategies for multiprotein complex assembly by co-expression: application to complexes as large as the histone octamer
ML Diebold, S Fribourg, M Koch, T Metzger, C Romier
Journal of structural biology 175 (2), 178-188, 2011
1322011
Inborn errors in RNA polymerase III underlie severe varicella zoster virus infections
B Ogunjimi, SY Zhang, KB Sørensen, KA Skipper, M Carter-Timofte, ...
The Journal of clinical investigation 127 (9), 3543-3556, 2017
1282017
RPS19 mutations in patients with Diamond‐Blackfan anemia
MF Campagnoli, U Ramenghi, M Armiraglio, P Quarello, E Garelli, ...
Human mutation 29 (7), 911-920, 2008
1212008
Dissecting the interaction network of multiprotein complexes by pairwise coexpression of subunits in E. coli
S Fribourg, C Romier, S Werten, YG Gangloff, A Poterszman, D Moras
Journal of molecular biology 306 (2), 363-373, 2001
892001
Peptides derived from the bifunctional kinase/RNase enzyme IRE1α modulate IRE1α activity and protect cells from endoplasmic reticulum stress
M Bouchecareilh, A Higa, S Fribourg, M Moenner, E Chevet
The FASEB Journal 25 (9), 3115-3129, 2011
882011
Molecular basis of Diamond–Blackfan anemia: structure and function analysis of RPS19
LA Gregory, AH Aguissa-Toure, N Pinaud, P Legrand, PE Gleizes, ...
Nucleic Acids Research 35 (17), 5913-5921, 2007
792007
Structural similarity in the absence of sequence homology of the messenger RNA export factors Mtr2 and p15
S Fribourg, E Conti
EMBO reports 4 (7), 699-703, 2003
682003
Structural and functional aspects of winged-helix domains at the core of transcription initiation complexes
M Teichmann, H Dumay-Odelot, S Fribourg
Transcription 3 (1), 2-7, 2012
592012
Exploring TAR–RNA aptamer loop–loop interaction by X-ray crystallography, UV spectroscopy and surface plasmon resonance
I Lebars, P Legrand, A Aimé, N Pinaud, S Fribourg, C Di Primo
Nucleic acids research 36 (22), 7146-7156, 2008
582008
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