MG132 Induces Progerin Clearance and Improves Disease Phenotypes in HGPS-like Patients’ Cells

Autor: Karim Harhouri, Pierre Cau, Frank Casey, Koffi Mawuse Guedenon, Yassamine Doubaj, Lionel Van Maldergem, Gerardo Mejia-Baltodano, Catherine Bartoli, Annachiara De Sandre-Giovannoli, Nicolas Lévy

Publikováno v: Cells, Vol 11, Iss 4, p 610 (2022)

Progeroid syndromes (PS), including Hutchinson-Gilford Progeria Syndrome (HGPS), are premature and accelerated aging diseases, characterized by clinical features mimicking physiological aging. Most classical HGPS patients carry a de novo point mutati

Externí odkaz: https://doaj.org/article/5b13b46a4b71430faf1e116cd2bbaac3

Antisense-Based Progerin Downregulation in HGPS-Like Patients’ Cells

Autor: Karim Harhouri, Claire Navarro, Camille Baquerre, Nathalie Da Silva, Catherine Bartoli, Frank Casey, Guedenon Koffi Mawuse, Yassamine Doubaj, Nicolas Lévy, Annachiara De Sandre-Giovannoli

Publikováno v: Cells, Vol 5, Iss 3, p 31 (2016)

Progeroid laminopathies, including Hutchinson-Gilford Progeria Syndrome (HGPS, OMIM #176670), are premature and accelerated aging diseases caused by defects in nuclear A-type Lamins. Most HGPS patients carry a de novo point mutation within exon 11 of

Externí odkaz: https://doaj.org/article/528d9dadacbd4b30b7e1ac3dea03165c

Antisense-Based Progerin Downregulation in HGPS-Like Patients’ Cells

Autor: Harhouri, Karim, Navarro, Claire, Baquerre, Camille, Da Silva, Nathalie, Bartoli, Catherine, Casey, Frank, Mawuse, Guedenon, Doubaj, Yassamine, Lévy, Nicolas, De Sandre-Giovannoli, Annachiara

Publikováno v: Cells
Cells, 2016, 5 (3), pp.31. ⟨10.3390/cells5030031⟩
Cells, MDPI, 2016, 5 (3), pp.31. ⟨10.3390/cells5030031⟩
Cells, Vol 5, Iss 3, p 31 (2016)

International audience; Progeroid laminopathies, including Hutchinson-Gilford Progeria Syndrome (HGPS, OMIM #176670), are premature and accelerated aging diseases caused by defects in nuclear A-type Lamins. Most HGPS patients carry a de novo point mu

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid_dedup__::cfcb7f679fcfd7881360b290b6aa74cb
https://hal-amu.archives-ouvertes.fr/hal-01769404/document

Antisense-Based Progerin Downregulation in HGPS-Like Patients' Cells.

Autor: Harhouri K; Aix Marseille Université, INSERM, GMGF UMR_S 910, 13385 Marseille, France. Karim.HARHOURI@univ-amu.fr., Navarro C; Aix Marseille Université, INSERM, GMGF UMR_S 910, 13385 Marseille, France. Claire.NAVARRO@univ-amu.fr., Baquerre C; Aix Marseille Université, INSERM, GMGF UMR_S 910, 13385 Marseille, France. camille.baquerre1@orange.fr., Da Silva N; Aix Marseille Université, INSERM, GMGF UMR_S 910, 13385 Marseille, France. Nathalie.DASILVA@univ-amu.fr., Bartoli C; Aix Marseille Université, INSERM, GMGF UMR_S 910, 13385 Marseille, France. catherine.bartoli@univ-amu.fr., Casey F; Royal Belfast, Pediatric Cardiology, Hospital for Sick Children, Belfast BT9 7AB, Northern Ireland. frank.casey@belfasttrust.hscni.net., Mawuse GK; CHU Sylvanus Olympio de Lomé, Unité de Génétique Humaine, Lomé BP 1515, Togo. julesblack@yahoo.fr., Doubaj Y; Département de Génétique Médicale, Institut National d'Hygiène, 11400 Rabat, Morocco. y.doubaj@gmail.com., Lévy N; Aix Marseille Université, INSERM, GMGF UMR_S 910, 13385 Marseille, France. nicolas.levy@univ-amu.fr.; Departement of Medical Genetics, la Timone Children's Hospital, APHM, 13385 Marseille, France. nicolas.levy@univ-amu.fr., De Sandre-Giovannoli A; Aix Marseille Université, INSERM, GMGF UMR_S 910, 13385 Marseille, France. annachiara.desandre-giovannoli@univ-amu.fr.; Departement of Medical Genetics, la Timone Children's Hospital, APHM, 13385 Marseille, France. annachiara.desandre-giovannoli@univ-amu.fr.

Publikováno v: Cells [Cells] 2016 Jul 11; Vol. 5 (3). Date of Electronic Publication: 2016 Jul 11.