Sébastien Fribourg

Molecular basis of Diamond Blackfan anemia: structure and function analysis of RPS19. (2007)

Gregory, Lynn, Aguissa-Touré, Almass-Houd, Pinaud, Noël, Legrand, Pierre, Gleizes, Pierre-Emmanuel, Fribourg, Sébastien

Diamond-Blackfan anemia (DBA) is a rare congenital disease linked to mutations in the ribosomal protein genes rps19, rps24 and rps17. It belongs to the emerging class of ribosomal disorders. To...

The structure of the CstF-77 homodimer provides insights into CstF assembly. (2007)

Legrand, Pierre, Pinaud, Noël, Minvielle-Sébastia, Lionel, Fribourg, Sébastien

The cleavage stimulation factor (CstF) is essential for the first step of poly(A) tail formation at the 3' ends of mRNAs. This heterotrimeric complex is built around the 77-kDa protein bridging both...

Molecular basis of Diamond Blackfan anemia: structure and function analysis of RPS19. (2007)

Gregory, Lynn, Aguissa-Touré, Almass-Houd, Pinaud, Noël, Legrand, Pierre, Gleizes, Pierre-Emmanuel, Fribourg, Sébastien

Diamond-Blackfan anemia (DBA) is a rare congenital disease linked to mutations in the ribosomal protein genes rps19, rps24 and rps17. It belongs to the emerging class of ribosomal disorders. To...

The structure of the CstF-77 homodimer provides insights into CstF assembly. (2007)

Legrand, Pierre, Pinaud, Noël, Minvielle-Sébastia, Lionel, Fribourg, Sébastien

The cleavage stimulation factor (CstF) is essential for the first step of poly(A) tail formation at the 3' ends of mRNAs. This heterotrimeric complex is built around the 77-kDa protein bridging both...

Structural similarity in the absence of sequence homology of the messenger RNA export factors Mtr2 and p15

Fribourg, Sébastien, Conti, Elena

The association between Mtr2 and Mex67 is essential for the nuclear export of bulk messenger RNA in yeast. In metazoans, the analogous function is carried out by the TAP–p15 heterodimer. Whereas...

Structural similarity in the absence of sequence homology of the messenger RNA export factors Mtr2 and p15

Fribourg, Sébastien, Conti, Elena

The association between Mtr2 and Mex67 is essential for the nuclear export of bulk messenger RNA in yeast. In metazoans, the analogous function is carried out by the TAP–p15 heterodimer. Whereas...

The structure of the CstF-77 homodimer provides insights into CstF assembly

Legrand, Pierre, Pinaud, Noël, Minvielle-Sébastia, Lionel, Fribourg, Sébastien

The cleavage stimulation factor (CstF) is essential for the first step of poly(A) tail formation at the 3' ends of mRNAs. This heterotrimeric complex is built around the 77-kDa protein bridging both...

Molecular basis of Diamond–Blackfan anemia: structure and function analysis of RPS19

Gregory, Lynn A., Aguissa-Touré, Almass-Houd, Pinaud, Noël, Legrand, Pierre, Gleizes, Pierre-Emmanuel, Fribourg, Sébastien

Diamond–Blackfan anemia (DBA) is a rare congenital disease linked to mutations in the ribosomal protein genes rps19, rps24 and rps17. It belongs to the emerging class of ribosomal disorders. To...

Impaired ribosome biogenesis in Diamond-Blackfan anemia

Choesmel, Valérie, Bacqueville, Daniel, Rouquette, Jacques, Noaillac-Depeyre, Jacqueline, Fribourg, Sébastien, Crétien, Aurore, ...

The gene encoding the ribosomal protein S19 (RPS19) is frequently mutated in Diamond-Blackfan anemia (DBA), a congenital erythroblastopenia. The consequence of these mutations on the onset of the...