[et_pb_section fb_built=\u201d1\u2033 _builder_version=\u201d3.22\u2033][et_pb_row _builder_version=\u201d3.25\u2033 background_size=\u201dinitial\u201d background_position=\u201dtop_left\u201d background_repeat=\u201dr\u00e9p\u00e9ter\u201d][et_pb_column type=\u201d4_4\u2033 _builder_version=\u201d3.25\u2033 custom_padding=\u201d|||\u201d custom_padding__hover=\u201d|||\u201d][et_pb_text _builder_version=\u201d4.6.5\u2033 background_size=\u201dinitial\u201d background_position=\u201dtop_left\u201d background_repeat=\u201dr\u00e9p\u00e9ter\u201d hover_enabled=\u201d0\u2033 sticky_enabled=\u201d0\u2033]<\/p>\n
Actes de la National Academy of Sciences, juillet 2005<\/em><\/p>\n * Par Shao H. Yang, Julia I. Toth, Yan Hu, Salemiz Sandoval, Stephen G. Young et Loren G. Fong, David Geffen School of Medicine, UCLA ; Margarita Meta, Universit\u00e9 de Californie, San Francisco ; Pravin Bendale et Michael H. Gelb, Universit\u00e9 de Washington, Seattle ; Martin O. Bergo, H\u00f4pital universitaire Sahlgrenska, Su\u00e8de<\/p>\n Apr\u00e8s avoir cr\u00e9\u00e9 un mod\u00e8le murin ciblant les g\u00e8nes du syndrome de Hutchinson-Gilford Progeria (HGPS), les auteurs ont cherch\u00e9 \u00e0 prouver que l'inhibition d'un processus appel\u00e9 farn\u00e9sylation des prot\u00e9ines par des inhibiteurs de la farn\u00e9syltransf\u00e9rase (FTI) peut bloquer les dommages caus\u00e9s \u00e0 l'enveloppe nucl\u00e9aire par la prot\u00e9ine mutante prog\u00e9rine. Les \u00e9tudes sugg\u00e8rent que les cellules peuvent \u00eatre r\u00e9par\u00e9es gr\u00e2ce \u00e0 cette approche.<\/p>\n La pr\u00e9lamine A mutante dans HGPS, commun\u00e9ment appel\u00e9e prog\u00e9rine, est caus\u00e9e par une mutation dans LMNA <\/em>cela entra\u00eene la suppression de 50 acides amin\u00e9s dans la pr\u00e9lamine A et emp\u00eache le traitement normal en lamine A mature. La pr\u00e9sence de prog\u00e9rine dans les cellules affecte n\u00e9gativement l'int\u00e9grit\u00e9 de la lame nucl\u00e9aire, ce qui entra\u00eene des noyaux d\u00e9form\u00e9s et des bulles nucl\u00e9aires.<\/p>\n Fong et son groupe ont examin\u00e9 les effets d\u2019un FTI sur ce processus et ont d\u00e9couvert qu\u2019il entra\u00eenait une am\u00e9lioration frappante de la forme nucl\u00e9aire (r\u00e9duction du nombre de noyaux d\u00e9form\u00e9s et endommag\u00e9s).<\/p>\n \u00ab Ces \u00e9tudes sugg\u00e8rent une strat\u00e9gie th\u00e9rapeutique possible pour la prog\u00e9ria \u00bb, d\u00e9clare le Dr Steven Young, co-auteur, \u00ab ce qui laisse esp\u00e9rer que les FTI pourraient finalement s\u2019av\u00e9rer utiles pour traiter la prog\u00e9ria. \u00bb<\/p>\n [\/et_pb_text][\/et_pb_column][\/et_pb_row][\/et_pb_section]<\/p>","protected":false},"excerpt":{"rendered":" [et_pb_section fb_built=”1″ _builder_version=”3.22″][et_pb_row _builder_version=”3.25″ background_size=”initial” background_position=”top_left” background_repeat=”repeat”][et_pb_column type=”4_4″ _builder_version=”3.25″ custom_padding=”|||” custom_padding__hover=”|||”][et_pb_text _builder_version=”4.6.5″ background_size=”initial” background_position=”top_left” background_repeat=”repeat” hover_enabled=”0″ sticky_enabled=”0″] Proceedings National Academy of Sciences, July 2005 * By Shao H. Yang, Julia I. Toth, Yan Hu, Salemiz Sandoval, Stephen G. Young, and Loren G. Fong, David Geffen School of Medicine, UCLA; Margarita Meta, University of California, San […]<\/p>","protected":false},"author":1,"featured_media":2043,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_et_pb_use_builder":"on","_et_pb_old_content":" [vc_custom_heading text=\"Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts containing a targeted Hutchinson-Gil\" font_container=\"tag:h1|text_align:left\" use_theme_fonts=\"yes\"]Blocking protein improves nuclear blebbing in mouse fibroblasts containing a targeted Hutchinson-Gilford Progeria syndrome mutation.<\/p> Proceedings National Academy of Sciences, July 2005<\/em><\/p> * By Shao H. Yang, Julia I. Toth, Yan Hu, Salemiz Sandoval, Stephen G. Young, and Loren G. Fong, David Geffen School of Medicine, UCLA; Margarita Meta, University of California, San Francisco; Pravin Bendale and Michael H. Gelb, University of Washington, Seattle; Martin O. Bergo, Sahlgrenska University Hospital, Sweden<\/p> After creating a gene-targeted mouse model of Hutchinson-Gilford Progeria Syndrome (HGPS), the authors set out to prove that the inhibition of a process called protein farnesylation with farnesyltransferase inhibitors (FTIs) can block the damage to the nuclear envelope caused by the mutant protein progerin. The studies suggest the cells can be repaired using this approach.<\/p> The mutant prelamin A in HGPS, commonly called progerin, is caused by a mutation in LMNA <\/em>that results in the deletion of 50 amino acids within prelamin A and prevents normal processing to mature lamin A. The presence of progerin in cells adversely affects the integrity of the nuclear lamina, resulting in misshapen nuclei and nuclear blebs.<\/p> Fong and his group examined the effects of an FTI on this process and found that it resulted in a striking improvement on nuclear shape (reduced misshapen and damaged nuclei).<\/p> \"These studies suggest a possible treatment strategy for Progeria\" says co-author Dr. Steven Young, \"raising the hope that FTIs might ultimately prove useful for treating Progeria.\"<\/p>","_et_gb_content_width":"","footnotes":"","_links_to":"","_links_to_target":""},"categories":[2],"tags":[],"class_list":["post-2130","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-news"],"yoast_head":"\n