{"id":1477,"date":"2017-04-19T12:43:11","date_gmt":"2017-04-19T12:43:11","guid":{"rendered":"https:\/\/www.progeriaresearch.org\/?page_id=1477"},"modified":"2023-09-21T11:28:47","modified_gmt":"2023-09-21T15:28:47","slug":"about-progeria","status":"publish","type":"page","link":"https:\/\/www.progeriaresearch.org\/de\/about-progeria\/","title":{"rendered":"\u00dcber Progerie"},"content":{"rendered":"<p>[et_pb_section fb_built=\u201d1\u2033 fullwidth=\u201don\u201d disabled_on=\u201doff|off|off\u201d _builder_version=\u201d4.16\u2033 border_width_bottom=\u201d55px\u201d border_color_bottom=\u201d#29327a\u201d global_colors_info=\u201d{}\u201d][et_pb_fullwidth_header _builder_version=\u201d4.16\u2033 title_font_size=\u201d55\u2033 background_color=\u201d#29327a\u201d background_image=\u201dhttps:\/\/www.progeriaresearch.org\/wp-content\/uploads\/2019\/04\/About-Header.jpg\u201d background_position=\u201dcenter_left\u201d custom_padding=\u201d9vw||9vw||true\u201d custom_padding_tablet=\u201d\u201d custom_padding_phone=\u201d|56px||\u201d custom_padding_last_edited=\u201dauf|Desktop\u201d title_font_size_tablet=\u201d45px\u201d title_font_size_phone=\u201d40px\u201d title_font_size_last_edited=\u201dauf|Telefon\u201d z_index_tablet=\u201d500\u2033 custom_css_main_element=\u201dHintergrundposition: Mitte 18% !wichtig;\u201d global_colors_info=\u201d{}\u201d]<\/p>\n<h1>\u00dcber Progerie<\/h1>\n<p>[\/et_pb_fullwidth_header][\/et_pb_section][et_pb_section fb_built=\u201d1\u2033 use_custom_gutter=\u201don\u201d gutter_width=\u201d1\u2033 specialty=\u201don\u201d padding_left_1=\u201d35px\u201d padding_left_2=\u201d35px\u201d padding_2_tablet=\u201d|||0px\u201d padding_2_phone=\u201d|||0px\u201d padding_2_last_edited=\u201don|desktop\u201d module_class_1=\u201dsidebar-secondary-nav\u201d module_class=\u201dhandprint-bg\u201d _builder_version=\u201d4.16\u2033 background_image=\u201dhttps:\/\/www.progeriaresearch.org\/wp-content\/uploads\/2019\/04\/blue-handprint-only.png\u201d parallax=\u201don\u201d parallax_method=\u201doff\u201d inner_width=\u201d100%\u201d inner_max_width=\u201d100%\u201d custom_padding=\u201d0|0px|54px|0px|false|false\u201d z_index_tablet=\u201d500\u2033 border_width_top=\u201d10px\u201d border_color_top=\u201d#8fd2ed\u201d use_custom_width=\u201dan\u201d width_unit=\u201daus\u201d custom_width_percent=\u201d100%\u201d global_colors_info=\u201d{}\u201d][et_pb_column type=\u201d1_4\u2033 _builder_version=\u201d4.16\u2033 custom_padding=\u201d|||\u201d global_colors_info=\u201d{}\u201d custom_padding__hover=\u201d|||\u201d][et_pb_sidebar-Bereich=\u201det_pb_widget_area_18\u2033 disabled_on=\u201dan|an|aus\u201d module_class=\u201dUnterseiten-Seitenleisten\u201d _builder_version=\u201d4.16\u2033 animation_style=\u201dverblassen\u201d z_index_tablet=\u201d500\u2033 border_width_right=\u201d5px\u201d global_colors_info=\u201d{}\u201d]<\/p>\n<p>[\/et_pb_sidebar][\/et_pb_column][et_pb_column Typ=\u201d3_4\u2033 Spezialspalten=\u201d3\u2033 _builder_version=\u201d4.16\u2033 benutzerdefiniertes_Padding=\u201d|||\u201d global_colors_info=\u201d{}\u201d custom_padding__hover=\u201d|||\u201d][et_pb_row_inner custom_padding_last_edited=\u201dam|Telefon\u201d _builder_version=\u201d4.16\u2033 custom_padding=\u201d39.4375px|35px|35px||false|false\u201d custom_padding_tablet=\u201d|35px||35px||true\u201d custom_padding_phone=\u201d\u201d animation_direction=\u201doben\u201d global_colors_info=\u201d{}\u201d][et_pb_column_inner saved_specialty_column_type=\u201d3_4\u2033 _builder_version=\u201d4.16\u2033 custom_padding=\u201d|||\u201d global_colors_info=\u201d{}\u201d custom_padding__hover=\u201d|||\u201d][et_pb_text _builder_version=\u201d4.19.5\u2033 Hintergrundgr\u00f6\u00dfe=\u201dinitial\u201d Hintergrundposition=\u201doben_links\u201d Hintergrundwiederholung=\u201dwiederholen\u201d global_colors_info=\u201d{}\u201d]<\/p>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"size-full wp-image-1479 alignleft\" src=\"https:\/\/www.progeriaresearch.org\/wp-content\/uploads\/2017\/04\/lindsay-Jul08-crpd.jpg\" alt=\"\" width=\"193\" height=\"164\" \/>Das Hutchinson-Gilford-Progerie-Syndrom (\u201eProgerie\u201c oder \u201eHGPS\u201c) ist eine seltene, t\u00f6dliche genetische Erkrankung, die durch beschleunigtes Altern bei Kindern gekennzeichnet ist. Der Name kommt aus dem Griechischen und bedeutet \u201evorzeitig alt werden\u201c. Es gibt zwar verschiedene Formen der Progerie*, der klassische Typ ist jedoch das Hutchinson-Gilford-Progerie-Syndrom, das nach den \u00c4rzten benannt wurde, die es in England erstmals beschrieben haben: 1886 von Dr. Jonathan Hutchinson und 1897 von Dr. Hastings Gilford.<\/p>\n<p>HGPS wird durch eine Mutation im Gen LMNA (ausgesprochen \u201eLamin \u2013 a\u201c) verursacht. Das LMNA-Gen produziert das Protein Lamin A, das strukturelle Ger\u00fcst, das den Zellkern zusammenh\u00e4lt. Forscher glauben heute, dass das defekte Protein Lamin A den Zellkern instabil macht. Diese Zellinstabilit\u00e4t scheint bei Progerie zum Prozess der vorzeitigen Alterung zu f\u00fchren.<\/p>\n<p>Obwohl sie bei der Geburt gesund aussehen, zeigen Kinder mit Progerie bereits in den ersten beiden Lebensjahren viele Anzeichen einer beschleunigten Alterung. Zu den Anzeichen von Progerie z\u00e4hlen Wachstumsst\u00f6rungen, Verlust von K\u00f6rperfett und Haaren, gealterte Haut, Gelenksteifheit, H\u00fcftgelenksverrenkung, generalisierte Arteriosklerose, Herz-Kreislauf-Erkrankungen und Schlaganfall. Die Kinder sehen trotz unterschiedlicher ethnischer Herkunft bemerkenswert \u00e4hnlich aus. Ohne Behandlung sterben Kinder mit Progerie im durchschnittlichen Alter von 14,5 Jahren an Arteriosklerose (Herzkrankheit).<\/p>\n<p>* Zu den weiteren progeroiden Syndromen geh\u00f6rt das Werner-Syndrom, auch bekannt als \u201eErwachsenen-Progerie\u201c, das erst in den sp\u00e4ten Teenagerjahren auftritt, die Lebenserwartung jedoch bis in die 40er und 50er Jahre reicht.<\/p>\n<p>[\/et_pb_text][et_pb_button button_url=\u201dhttps:\/\/www.progeriaresearch.org\/scientific-publications\/\u201d button_text=\u201dWissenschaftliche Publikationen anzeigen\u201d _builder_version=\u201d4.16\u2033 background_layout=\u201ddark\u201d custom_margin=\u201d20px||\u201d global_colors_info=\u201d{}\u201d]<\/p>\n<p>[\/et_pb_button][\/et_pb_column_inner][\/et_pb_row_inner][\/et_pb_column][\/et_pb_section][et_pb_section fb_built=\u201d1\u2033 module_class=\u201dfooter\u201d _builder_version=\u201d4.21.0\u2033 background_color=\u201d#29327a\u201d custom_margin=\u201d-2px|||||\u201d custom_padding=\u201d0|0px|0|0px|false|false\u201d z_index_tablet=\u201d500\u2033 border_width_top=\u201d12px\u201d border_color_top=\u201d#00b2e2\u2033 global_module=\u201d133\u2033 gesperrt=\u201daus\u201d global_colors_info=\u201d{}\u201d][et_pb_row column_structure=\u201d1_4,1_4,1_2\u2033 make_equal=\u201dan\u201d module_class=\u201d et_pb_row_fullwidth\u201d _builder_version=\u201d4.16\u2033 width=\u201d89%\u201d width_tablet=\u201d80%\u201d width_phone=\u201d\u201d width_last_edited=\u201dan|desktop\u201d max_width=\u201d89%\u201d max_width_tablet=\u201d80%\u201d max_width_phone=\u201d\u201d max_width_last_edited=\u201dan|desktop\u201d z_index_tablet=\u201d500\u2033 make_fullwidth=\u201dan\u201d width_unit=\u201daus\u201d custom_width_percent=\u201d100%\u201d global_colors_info=\u201d{}\u201d][et_pb_column type=\u201d1_4\u2033 _builder_version=\u201d4.16\u2033 custom_padding=\u201d|||\u201d global_colors_info=\u201d{}\u201d custom_padding__hover=\u201d|||\u201d][et_pb_cta button_url=\u201dhttps:\/\/www.progeriaresearch.org\/newsletter-signup\/\u201d button_text=\u201dJetzt anmelden\u201d admin_label=\u201dF\u00fcr Newsletter anmelden\u201d module_class=\u201dsign-btn\u201d _builder_version=\u201d4.16\u2033 header_font_size=\u201d25px\u201d background_color=\u201d#29327a\u201d animation_style=\u201dslide\u201d animation_direction=\u201dleft\u201d animation_intensity_slide=\u201d25%\u201d header_font_size_tablet=\u201d\u201d header_font_size_phone=\u201d30px\u201d header_font_size_last_edited=\u201don|desktop\u201d z_index_tablet=\u201d500\u2033 border_radii=\u201dan|25px|25px|25px|25px\u201d global_colors_info=\u201d{}\u201d button_bg_color__hover_enabled=\u201dan\u201d button_bg_color__hover=\u201d#8fd2ed\u201d button_border_color__hover_enabled=\u201dan\u201d]<\/p>\n<h2>Melden Sie sich an<\/h2>\n<h2>f\u00fcr unsere<\/h2>\n<h2>Newsletter!<\/h2>\n<p>[\/et_pb_cta][\/et_pb_column][et_pb_column type=\u201d1_4\u2033 _builder_version=\u201d4.16\u2033 custom_padding=\u201d|||\u201d global_colors_info=\u201d{}\u201d custom_padding__hover=\u201d|||\u201d][et_pb_cta button_url=\u201dhttps:\/\/progeriaresearch.donorsupport.co\/-\/XZHJVWZR\u201d button_text=\u201dJetzt spenden\u201d admin_label=\u201dGemeinsam werden wir die Heilung finden!\u201d module_class=\u201dsign-btn\u201d _builder_version=\u201d4.16\u2033 header_font_size=\u201d25px\u201d background_color=\u201d#29327a\u201d animation_style=\u201dslide\u201d animation_direction=\u201dleft\u201d animation_intensity_slide=\u201d25%\u201d header_font_size_tablet=\u201d\u201d header_font_size_phone=\u201d30px\u201d header_font_size_last_edited=\u201don|desktop\u201d body_font_size_tablet=\u201d\u201d body_font_size_phone=\u201d\u201d body_font_size_last_edited=\u201don|desktop\u201d z_index_tablet=\u201d500\u2033 border_radii=\u201don|25px|25px|25px|25px\u201d global_colors_info=\u201d{}\u201d button_bg_color__hover_enabled=\u201don\u201d button_bg_color__hover=\u201d#8fd2ed\u201d button_border_color__hover_enabled=\u201dein\u201d]<\/p>\n<h2>Gemeinsam<\/h2>\n<h2><em>WILLE<\/em><\/h2>\n<h2>Finde die Heilung!<\/h2>\n<p>[\/et_pb_cta][\/et_pb_column][et_pb_column Typ=\u201d1_2\u2033 _builder_version=\u201d4.16\u2033 custom_padding=\u201d|||\u201d global_colors_info=\u201d{}\u201d custom_padding__hover=\u201d|||\u201d][et_pb_image src=\u201dhttps:\/\/www.progeriaresearch.org\/wp-content\/uploads\/2023\/06\/2023-footer-strip-5.png\u201d title_text=\u201d2023 Fu\u00dfzeilenstreifen 5\u2033 _builder_version=\u201d4.21.0\u2033 _module_preset=\u201ddefault\u201d custom_margin=\u201d35px||||false|false\u201d global_colors_info=\u201d{}\u201d][\/et_pb_image][\/et_pb_column][\/et_pb_row][\/et_pb_section]<\/p>","protected":false},"excerpt":{"rendered":"<p>[et_pb_section fb_built=\u201d1\u2033 fullwidth=\u201don\u201d disabled_on=\u201doff|off|off\u201d _builder_version=\u201d4.16\u2033 border_width_bottom=\u201d55px\u201d border_color_bottom=\u201d#29327a\u201d global_colors_info=\u201d{}\u201d][et_pb_fullwidth_header _builder_version=\u201d4.16\u2033 title_font_size=\u201d55\u2033 background_color=\u201d#29327a\u201d background_image=\u201dhttps:\/\/www.progeriaresearch.org\/wp-content\/uploads\/2019\/04\/About-Header.jpg\u201d background_position=\u201dcenter_left\u201d custom_padding=\u201d9vw||9vw||true\u201d custom_padding_tablet=\u201d\u201d custom_padding_phone=\u201d|56px||\u201d custom_padding_last_edited=\u201dauf|Desktop\u201d title_font_size_tablet=\u201d45px\u201d title_font_size_phone=\u201d40px\u201d title_font_size_last_edited=\u201dauf|Telefon\u201d z_index_tablet=\u201d500\u2033 custom_css_main_element=\u201dHintergrundposition: Mitte 18% !wichtig;\u201d global_colors_info=\u201d{}\u201d] \u00dcber Progerie [\/et_pb_fullwidth_header][\/et_pb_section][et_pb_section fb_built=\u201d1\u2033 use_custom_gutter=\u201don\u201d gutter_width=\u201d1\u2033 specialty=\u201don\u201d padding_left_1=\u201d35px\u201d padding_left_2=\u201d35px\u201d padding_2_tablet=\u201d|||0px\u201d padding_2_phone=\u201d|||0px\u201d padding_2_last_edited=\u201don|desktop\u201d module_class_1=\u201dsidebar-secondary-nav\u201d module_class=\u201dhandprint-bg\u201d _builder_version=\u201d4.16\u2033 background_image=\u201dhttps:\/\/www.progeriaresearch.org\/wp-content\/uploads\/2019\/04\/blue-handprint-only.png\u201d parallax=\u201don\u201d parallax_method=\u201daus\u201d inner_width=\u201d100%\u201d inner_max_width=\u201d100%\u201d custom_padding=\u201d0|0px|54px|0px|false|false\u201d z_index_tablet=\u201d500\u2033 border_width_top=\u201d10px\u201d border_color_top=\u201d#8fd2ed\u201d use_custom_width=\u201dan\u201d width_unit=\u201daus\u201d custom_width_percent=\u201d100%\u201d global_colors_info=\u201d{}\u201d][et_pb_column type=\u201d1_4\u2033 [\u2026]<\/p>","protected":false},"author":1,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_et_pb_use_builder":"on","_et_pb_old_content":"\t\t\t\t[vc_row][vc_column][vc_custom_heading text=\"About Progeria\" font_container=\"tag:h1|text_align:center\" use_theme_fonts=\"yes\"][vc_column_text]<img class=\"size-full wp-image-1479 alignleft\" src=\"https:\/\/www.progeriaresearch.org\/wp-content\/uploads\/2017\/04\/lindsay-Jul08-crpd.jpg\" alt=\"\" width=\"193\" height=\"164\" \/>Hutchinson-Gilford Progeria Syndrome (\"Progeria\", or \"HGPS\") is a rare, fatal genetic condition characterized by an appearance of accelerated aging in children. Its name is derived from the Greek and means \"prematurely old.\" While there are different forms of Progeria*, the classic type is Hutchinson-Gilford Progeria Syndrome, which was named after the doctors who first described it in England; in 1886 by Dr. Jonathan Hutchinson and in 1897 by Dr. Hastings Gilford.\r\n\r\nHGPS is caused by a mutation in the gene called LMNA (pronounced, lamin - a). The LMNA gene produces the Lamin A protein, which is the structural scaffolding that holds the nucleus of a cell together. Researchers now believe that the defective Lamin A protein makes the nucleus unstable. That cellular instability appears to lead to the process of premature aging in Progeria.\r\n\r\nAlthough they are born looking healthy, children with Progeria begin to display many characteristics of accelerated aging within the first two years of life.\u00a0Progeria signs include growth failure, loss of body fat and hair, aged-looking skin, stiffness of joints, hip dislocation, generalized atherosclerosis, cardiovascular (heart) disease and stroke. The children have a remarkably similar appearance, despite differing ethnic backgrounds. Children with Progeria die of atherosclerosis (heart disease) at an average age of fourteen years.\r\n\r\n* Other progeroid syndromes include Werner's syndrome, also known as \"adult progeria\" which does not have an onset until the late teen years, with a life span into the 40's and 50's.\r\n\r\n<a href=\"https:\/\/www.progeriaresearch.org\/scientific-publications\/\">Click here<\/a> for\u00a0information on\u00a0scientific articles on Progeria.[\/vc_column_text][\/vc_column][\/vc_row]\t\t","_et_gb_content_width":"","footnotes":"","_links_to":"","_links_to_target":""},"class_list":["post-1477","page","type-page","status-publish","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v26.8 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>About Progeria | The Progeria Research Foundation<\/title>\n<meta name=\"description\" content=\"Progeria is a rare, fatal genetic condition characterized by an appearance of accelerated aging in children. 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