[et_pb_section fb_built=\u201d1\u2033 _builder_version=\u201d3.22\u2033][et_pb_row _builder_version=\u201d3.25\u2033 background_size=\u201dinicial\u201d background_position=\u201dtopo_esquerdo\u201d background_repeat=\u201drepetir\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=\u201dinicial\u201d background_position=\u201dtopo_esquerdo\u201d background_repeat=\u201drepetir\u201d hover_enabled=\u201d0\u2033 sticky_enabled=\u201d0\u2033]<\/p>\n
Anais da Academia Nacional de Ci\u00eancias, julho de 2005<\/em><\/p>\n * Por Shao H. Yang, Julia I. Toth, Yan Hu, Salemiz Sandoval, Stephen G. Young e Loren G. Fong, Escola de Medicina David Geffen, UCLA; Margarita Meta, Universidade da Calif\u00f3rnia, S\u00e3o Francisco; Pravin Bendale e Michael H. Gelb, Universidade de Washington, Seattle; Martin O. Bergo, Hospital Universit\u00e1rio Sahlgrenska, Su\u00e9cia<\/p>\n Ap\u00f3s criar um modelo de camundongo de S\u00edndrome de Hutchinson-Gilford Progeria (HGPS) direcionado a genes, os autores se propuseram a provar que a inibi\u00e7\u00e3o de um processo chamado farnesila\u00e7\u00e3o de prote\u00ednas com inibidores de farnesiltransferase (FTIs) pode bloquear os danos ao envelope nuclear causados pela prote\u00edna mutante progerina. Os estudos sugerem que as c\u00e9lulas podem ser reparadas usando essa abordagem.<\/p>\n A prel\u00e2mina A mutante em HGPS, comumente chamada de progerina, \u00e9 causada por uma muta\u00e7\u00e3o em LMNA <\/em>que resulta na exclus\u00e3o de 50 amino\u00e1cidos na pr\u00e9-l\u00e2mina A e impede o processamento normal para a l\u00e2mina A madura. A presen\u00e7a de progerina nas c\u00e9lulas afeta negativamente a integridade da l\u00e2mina nuclear, resultando em n\u00facleos deformados e bolhas nucleares.<\/p>\n Fong e seu grupo examinaram os efeitos de um FTI nesse processo e descobriram que isso resultou em uma melhora impressionante no formato nuclear (redu\u00e7\u00e3o de n\u00facleos deformados e danificados).<\/p>\n \u201cEsses estudos sugerem uma poss\u00edvel estrat\u00e9gia de tratamento para a Prog\u00e9ria\u201d, diz o coautor Dr. Steven Young, \u201caumentando a esperan\u00e7a de que os FTIs possam, em \u00faltima an\u00e1lise, ser \u00fateis para tratar a Prog\u00e9ria\u201d.<\/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