{"id":2135,"date":"2005-05-16T20:48:59","date_gmt":"2005-05-16T20:48:59","guid":{"rendered":"https:\/\/www.progeriaresearch.org\/?p=2135"},"modified":"2020-12-09T10:24:44","modified_gmt":"2020-12-09T15:24:44","slug":"reversal-of-the-cellular-phenotype-in-the-premature-aging-disease-hgps","status":"publish","type":"post","link":"https:\/\/www.progeriaresearch.org\/de\/2005\/05\/16\/reversal-of-the-cellular-phenotype-in-the-premature-aging-disease-hgps\/","title":{"rendered":"Umkehrung des zellul\u00e4ren Ph\u00e4notyps bei der vorzeitigen Alterungskrankheit HGPS"},"content":{"rendered":"<p>[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=\u201dwiederholen\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=\u201dwiederholen\u201d hover_enabled=\u201d0\u2033 sticky_enabled=\u201d0\u2033]<\/p>\n<p>Zellen von Patienten mit Hutchinson-Gilford-Progerie-Syndrom (HGPS) k\u00f6nnen wieder gesund gemacht werden. Dies ist das Ergebnis der Erkenntnisse von Wissenschaftlern des National Cancer Institute, das zu den National Institutes of Health geh\u00f6rt.<\/p>\n<p><em>Online ver\u00f6ffentlicht am 6. M\u00e4rz 2005 in Nature Medicine <a href=\"https:\/\/www.nature.com\/\" target=\"_blank\" rel=\"noopener noreferrer\">www.nature.com<\/a><\/em><\/p>\n<p>Zellen von Patienten mit Hutchinson-Gilford-Progerie-Syndrom (HGPS) k\u00f6nnen wieder gesund gemacht werden, so die Erkenntnisse von Wissenschaftlern des National Cancer Institute, einem Teil der National Institutes of Health. Mithilfe speziell modifizierter kurzer DNA-Segmente konnten die NCI-Forscher Dr. Paola Scaffidi und Dr. Tom Misteli (beide Teilnehmer des PRF-Workshops 2003) die in HGPS-Zellen beobachteten Defekte r\u00fcckg\u00e4ngig machen, indem sie das bei HGPS fehlerhafte Lamin-A-Protein eliminierten. Indem diese Studie zeigt, dass zellul\u00e4re Ph\u00e4notypen von HGPS reversibel sind, bringt sie die Wissenschaftler der Heilung dieser verheerenden Kinderkrankheit einen Schritt n\u00e4her.<\/p>\n<p>\u201eWir wollten herausfinden, ob diese mit Progerie verbundenen Zellver\u00e4nderungen dauerhaft sind oder r\u00fcckg\u00e4ngig gemacht werden k\u00f6nnen\u201c, sagte Scaffidi. Die Forscher entwickelten ein \u201emolekulares Pflaster\u201c, sagte Misteli (ein chemisch stabiles DNA-Oligonukleotid, das die Zelle nicht abbauen kann). Eine Woche sp\u00e4ter war das mutierte Lamin-A-Protein eliminiert und \u00fcber 90 Prozent der Progeriezellen sahen normal aus. Auch die Aktivit\u00e4t mehrerer Gene, die bei HGPS-Patienten fehlreguliert sind, war wieder normal. \u201eEs ist erstaunlich, dass wir eine kranke Zelle nehmen konnten, die ein paar Tage sp\u00e4ter wieder gesund und bereit ist, sich wieder zu teilen\u201c, sagte Misteli.<\/p>\n<p>Er wies darauf hin, dass diese Ergebnisse einen prinzipiellen Beweis daf\u00fcr darstellten, dass die zellul\u00e4ren Auswirkungen der Progerie r\u00fcckg\u00e4ngig gemacht werden k\u00f6nnen, was bedeutet, dass die Methode seines Labors eines Tages als therapeutische Strategie eingesetzt werden k\u00f6nnte.<\/p>\n<p><strong>Ein Mangel an \u201egutem\u201c Cholesterin bei Kindern mit Progerie kann zu vorzeitiger Herzerkrankung beitragen.<\/strong> Adiponektin \u2013 ein Hormon, das den Fett- und Zuckerstoffwechsel reguliert \u2013 k\u00f6nnte bei der Suche nach Behandlungsm\u00f6glichkeiten hilfreich sein.<br \/><em>The Journal of Pediatrics, M\u00e4rz 2005<\/em><\/p>\n<p>Ein Forscherteam unter der Leitung des medizinischen Direktors der PRF und Assistenzprofessors Leslie Gordon, MD, PhD der Tufts University School of Medicine, hat herausgefunden, dass Kinder mit Progerie im mittleren und sp\u00e4teren Lebensalter geringere Werte von HDL-Cholesterin \u2013 oder \u201esch\u00fctzendem\u201c Cholesterin \u2013 und Adiponektin aufweisen, einem Hormon, das den Fett- und Zuckerstoffwechsel reguliert. Beide Faktoren wirken, indem sie Fett aus Plaques in den Arterien entfernen, und die niedrigeren Werte k\u00f6nnen zu einer beschleunigten Plaquebildung beitragen. \u201eAlle Kinder mit Progerie sterben im Alter zwischen 6 und 20 Jahren an Herzversagen oder Schlaganfall\u201c, sagte Dr. Gordon. \u201eDie Erforschung von Herzerkrankungen im Zusammenhang mit Kindern mit Progerie kann uns helfen, besser zu verstehen, wie sich Arteriosklerose auf die alternde Bev\u00f6lkerung auswirkt, und gleichzeitig diesen wertvollen Kindern helfen.\u201c<\/p>\n<p>\u201eDiese Erkenntnisse sind wichtig f\u00fcr die m\u00f6gliche Entwicklung einer Behandlung f\u00fcr Progerie\u201c, sagte der leitende Studienautor<strong> Alice H. Lichtenstein, DSc, Forschungszentrum f\u00fcr menschliche Ern\u00e4hrung im Altern an der Tufts University. <\/strong>\u201eWenn zuverl\u00e4ssige Medikamente zur Erh\u00f6hung des HDL-Cholesterin- und Adiponektinspiegels verf\u00fcgbar werden, k\u00f6nnten sie sich als hilfreich erweisen, das Fortschreiten der Arteriosklerose bei Kindern mit Progerie zu verhindern.\u201c<\/p>\n<p>\u201eDiese Daten unterstreichen auch die Bedeutung des Fettgewebes als aktives Gewebe, das Hormone absondert, die die Stoffwechselfunktion des gesamten K\u00f6rpers beeinflussen k\u00f6nnen \u2013 ein Konzept, das nicht nur f\u00fcr Progerie, sondern auch f\u00fcr h\u00e4ufigere Krankheiten wie Fettleibigkeit und Diabetes wichtig ist\u201c, kommentiert der Autor <strong>Mary Elizabeth Patti, MD, Joslin Diabetes Center und Abteilung f\u00fcr Medizin, Harvard Medical School, Boston, MA<\/strong><\/p>\n<p>[\/et_pb_text][\/et_pb_column][\/et_pb_row][\/et_pb_section]<\/p>","protected":false},"excerpt":{"rendered":"<p>[et_pb_section fb_built=&#8221;1&#8243; _builder_version=&#8221;3.22&#8243;][et_pb_row _builder_version=&#8221;3.25&#8243; background_size=&#8221;initial&#8221; background_position=&#8221;top_left&#8221; background_repeat=&#8221;repeat&#8221;][et_pb_column type=&#8221;4_4&#8243; _builder_version=&#8221;3.25&#8243; custom_padding=&#8221;|||&#8221; custom_padding__hover=&#8221;|||&#8221;][et_pb_text _builder_version=&#8221;4.6.5&#8243; background_size=&#8221;initial&#8221; background_position=&#8221;top_left&#8221; background_repeat=&#8221;repeat&#8221; hover_enabled=&#8221;0&#8243; sticky_enabled=&#8221;0&#8243;] Cells from patients with Hutchinson-Gilford Progeria Syndrome (HGPS) can be made healthy again, according to findings by scientists at the National Cancer Institute, part of the National Institutes of Health. Published online on March 6, 2005 in Nature [&hellip;]<\/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":"<p>[vc_custom_heading text=\"Reversal of the cellular phenotype in the premature aging disease HGPS\" font_container=\"tag:h1|text_align:left\" use_theme_fonts=\"yes\"]<\/p><div id=\"secondary-header-content\"><div id=\"secondary-header-content\"><p>Cells from patients with Hutchinson-Gilford Progeria Syndrome (HGPS) can be made healthy again, according to findings by scientists at the National Cancer Institute, part of the National Institutes of Health.<\/p><\/div><\/div><p><em>Published online on March 6, 2005 in Nature Medicine <a href=\"https:\/\/www.nature.com\/\">(www.nature.com)<\/a><\/em><\/p><p>Cells from patients with Hutchinson-Gilford Progeria Syndrome (HGPS) can be made healthy again, according to findings by scientists at the National Cancer Institute, part of the National Institutes of Health. Using specially modified short segments of DNA, NCI researchers Paola Scaffidi, PhD, and Tom Misteli, PhD (both 2003 PRF Workshop participants), reversed the defects seen in HGPS cells by eliminating the lamin A protein that is faulty in HGPS. By demonstrating that HGPS cellular phenotypes are reversible, this study brings scientists one step closer to curing this devastating childhood disease.<\/p><p>\"We set out to ask whether these cellular changes associated with Progeria are permanent or can be reversed,\" said Scaffidi. The researchers designed a \"molecular Band-Aid\u00ae,\" said Misteli (a chemically stable DNA oligonucleotide -so the cell wouldn't be able to degrade it.) One week later, the mutant lamin A protein had been eliminated and more than 90 percent of progeria cells looked normal; and the activity of several genes that are misregulated in HGPS patients were also restored to normal. \"It's amazing that we could take a sick cell and a few days later it's healthy and ready to divide again,\" said Misteli.<\/p><p>He noted that these results demonstrate a proof-of-principle that the cellular effects of Progeria can be reversed, meaning his laboratory's method might someday be used as a therapeutic strategy.<\/p><p><strong>Decreased levels of \"good\" cholesterol in children with Progeria may contribute to premature heart disease.<\/strong> Adiponectin - a hormone that regulates the metabolism of fat and sugar - may be helpful in finding treatments.<br \/><em>The Journal of Pediatrics, March 2005<\/em><\/p><p>A team of researchers led by PRF's Medical Director and Tufts University School of Medicine Assistant Professor Leslie Gordon, MD, PhD, discovered that children with Progeria in their mid- and later years have decreased levels of HDL cholesterol - or \"protective\" cholesterol\" - and adiponectin, a hormone that regulates the metabolism of fat and sugar. Both factors work to remove fat from plaques in arteries, and the lower levels may contribute to accelerated plaque formation. \"All children with Progeria die between the ages of 6 and 20 years from heart failure or stroke,\" said Dr. Gordon. \"Studying heart disease as it relates to children with Progeria can help us better understand how atherosclerosis will affect the aging population while also helping these precious children.\"<\/p><p>\"These findings are important for the potential development of a treatment for Progeria,\" said senior study author<strong> Alice H. Lichtenstein, DSc, Human Nutrition Research Center on Aging at Tufts University. <\/strong>\"If reliable drugs used to increase HDL cholesterol and adiponectin levels become available, they may prove beneficial in preventing the progression of atherosclerosis in children with Progeria.\"<\/p><p>\"These data also reinforce the importance of adipose tissue as an active tissue which secretes hormones which can influence metabolic function of the entire body - a concept important not only for Progeria but also for more common diseases such as obesity and diabetes\", comments author <strong>Mary Elizabeth Patti, MD, Joslin Diabetes Center, and Department of Medicine, Harvard Medical School, Boston, MA<\/strong><\/p>","_et_gb_content_width":"","footnotes":"","_links_to":"","_links_to_target":""},"categories":[2],"tags":[],"class_list":["post-2135","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-news"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v26.8 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Reversal of the cellular phenotype in the premature aging disease HGPS - The Progeria Research Foundation<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/www.progeriaresearch.org\/de\/2005\/05\/16\/reversal-of-the-cellular-phenotype-in-the-premature-aging-disease-hgps\/\" \/>\n<meta property=\"og:locale\" content=\"de_DE\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Reversal of the cellular phenotype in the premature aging disease HGPS - The Progeria Research Foundation\" \/>\n<meta property=\"og:description\" content=\"[et_pb_section fb_built=&#8221;1&#8243; _builder_version=&#8221;3.22&#8243;][et_pb_row _builder_version=&#8221;3.25&#8243; background_size=&#8221;initial&#8221; background_position=&#8221;top_left&#8221; background_repeat=&#8221;repeat&#8221;][et_pb_column type=&#8221;4_4&#8243; _builder_version=&#8221;3.25&#8243; custom_padding=&#8221;|||&#8221; custom_padding__hover=&#8221;|||&#8221;][et_pb_text _builder_version=&#8221;4.6.5&#8243; background_size=&#8221;initial&#8221; background_position=&#8221;top_left&#8221; background_repeat=&#8221;repeat&#8221; hover_enabled=&#8221;0&#8243; sticky_enabled=&#8221;0&#8243;] Cells from patients with Hutchinson-Gilford Progeria Syndrome (HGPS) can be made healthy again, according to findings by scientists at the National Cancer Institute, part of the National Institutes of Health. Published online on March 6, 2005 in Nature [&hellip;]\" \/>\n<meta property=\"og:url\" content=\"https:\/\/www.progeriaresearch.org\/de\/2005\/05\/16\/reversal-of-the-cellular-phenotype-in-the-premature-aging-disease-hgps\/\" \/>\n<meta property=\"og:site_name\" content=\"The Progeria Research Foundation\" \/>\n<meta property=\"article:publisher\" content=\"https:\/\/www.facebook.com\/ProgeriaResearch\/\" \/>\n<meta property=\"article:published_time\" content=\"2005-05-16T20:48:59+00:00\" \/>\n<meta property=\"article:modified_time\" content=\"2020-12-09T15:24:44+00:00\" \/>\n<meta property=\"og:image\" content=\"https:\/\/www.progeriaresearch.org\/wp-content\/uploads\/2011\/05\/1PRFlogo2010_300dpi.jpg\" \/>\n\t<meta property=\"og:image:width\" content=\"400\" \/>\n\t<meta property=\"og:image:height\" content=\"165\" \/>\n\t<meta property=\"og:image:type\" content=\"image\/jpeg\" \/>\n<meta name=\"author\" content=\"gravoc\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:creator\" content=\"@Progeria\" \/>\n<meta name=\"twitter:site\" content=\"@Progeria\" \/>\n<meta name=\"twitter:label1\" content=\"Written by\" \/>\n\t<meta name=\"twitter:data1\" content=\"gravoc\" \/>\n\t<meta name=\"twitter:label2\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data2\" content=\"3 Minuten\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\/\/schema.org\",\"@graph\":[{\"@type\":\"Article\",\"@id\":\"https:\/\/www.progeriaresearch.org\/ru\/2005\/05\/16\/reversal-of-the-cellular-phenotype-in-the-premature-aging-disease-hgps\/#article\",\"isPartOf\":{\"@id\":\"https:\/\/www.progeriaresearch.org\/ru\/2005\/05\/16\/reversal-of-the-cellular-phenotype-in-the-premature-aging-disease-hgps\/\"},\"author\":{\"name\":\"gravoc\",\"@id\":\"https:\/\/www.progeriaresearch.org\/ta\/#\/schema\/person\/a3c935a81e92242c6a77ae0018542928\"},\"headline\":\"Reversal of the cellular phenotype in the premature aging disease HGPS\",\"datePublished\":\"2005-05-16T20:48:59+00:00\",\"dateModified\":\"2020-12-09T15:24:44+00:00\",\"mainEntityOfPage\":{\"@id\":\"https:\/\/www.progeriaresearch.org\/ru\/2005\/05\/16\/reversal-of-the-cellular-phenotype-in-the-premature-aging-disease-hgps\/\"},\"wordCount\":620,\"commentCount\":0,\"publisher\":{\"@id\":\"https:\/\/www.progeriaresearch.org\/ta\/#organization\"},\"image\":{\"@id\":\"https:\/\/www.progeriaresearch.org\/ru\/2005\/05\/16\/reversal-of-the-cellular-phenotype-in-the-premature-aging-disease-hgps\/#primaryimage\"},\"thumbnailUrl\":\"https:\/\/www.progeriaresearch.org\/wp-content\/uploads\/2011\/05\/1PRFlogo2010_300dpi.jpg\",\"articleSection\":[\"News\"],\"inLanguage\":\"de\",\"potentialAction\":[{\"@type\":\"CommentAction\",\"name\":\"Comment\",\"target\":[\"https:\/\/www.progeriaresearch.org\/ru\/2005\/05\/16\/reversal-of-the-cellular-phenotype-in-the-premature-aging-disease-hgps\/#respond\"]}]},{\"@type\":\"WebPage\",\"@id\":\"https:\/\/www.progeriaresearch.org\/ru\/2005\/05\/16\/reversal-of-the-cellular-phenotype-in-the-premature-aging-disease-hgps\/\",\"url\":\"https:\/\/www.progeriaresearch.org\/ru\/2005\/05\/16\/reversal-of-the-cellular-phenotype-in-the-premature-aging-disease-hgps\/\",\"name\":\"Reversal of the cellular phenotype in the premature aging disease HGPS - 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The Progeria Research Foundation","robots":{"index":"index","follow":"follow","max-snippet":"max-snippet:-1","max-image-preview":"max-image-preview:large","max-video-preview":"max-video-preview:-1"},"canonical":"https:\/\/www.progeriaresearch.org\/de\/2005\/05\/16\/reversal-of-the-cellular-phenotype-in-the-premature-aging-disease-hgps\/","og_locale":"de_DE","og_type":"article","og_title":"Reversal of the cellular phenotype in the premature aging disease HGPS - The Progeria Research Foundation","og_description":"[et_pb_section fb_built=&#8221;1&#8243; _builder_version=&#8221;3.22&#8243;][et_pb_row _builder_version=&#8221;3.25&#8243; background_size=&#8221;initial&#8221; background_position=&#8221;top_left&#8221; background_repeat=&#8221;repeat&#8221;][et_pb_column type=&#8221;4_4&#8243; _builder_version=&#8221;3.25&#8243; custom_padding=&#8221;|||&#8221; custom_padding__hover=&#8221;|||&#8221;][et_pb_text _builder_version=&#8221;4.6.5&#8243; background_size=&#8221;initial&#8221; background_position=&#8221;top_left&#8221; background_repeat=&#8221;repeat&#8221; hover_enabled=&#8221;0&#8243; sticky_enabled=&#8221;0&#8243;] Cells from patients with Hutchinson-Gilford Progeria Syndrome (HGPS) can be made healthy again, according to findings by scientists at the National Cancer Institute, part of the National Institutes of Health. 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