{"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\/nl\/2005\/05\/16\/reversal-of-the-cellular-phenotype-in-the-premature-aging-disease-hgps\/","title":{"rendered":"Omkering van het cellulaire fenotype bij de vroegtijdige verouderingsziekte 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=\u201dherhalen\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=\u201dherhalen\u201d hover_enabled=\u201d0\u2033 sticky_enabled=\u201d0\u2033]<\/p>\n<p>Cellen van pati\u00ebnten met het Hutchinson-Gilford Progeria-syndroom (HGPS) kunnen weer gezond worden gemaakt, zo blijkt uit bevindingen van wetenschappers van het National Cancer Institute, onderdeel van de National Institutes of Health.<\/p>\n<p><em>Gepubliceerd online op 6 maart 2005 in Nature Medicine <a href=\"https:\/\/www.nature.com\/\" target=\"_blank\" rel=\"noopener noreferrer\">www.natuur.com<\/a><\/em><\/p>\n<p>Cellen van pati\u00ebnten met Hutchinson-Gilford Progeria Syndrome (HGPS) kunnen weer gezond worden gemaakt, volgens bevindingen van wetenschappers van het National Cancer Institute, onderdeel van de National Institutes of Health. Met behulp van speciaal aangepaste korte DNA-segmenten, keerden NCI-onderzoekers Paola Scaffidi, PhD, en Tom Misteli, PhD (beide deelnemers aan de PRF Workshop 2003) de defecten terug die werden gezien in HGPS-cellen door het lamin A-eiwit te elimineren dat defect is in HGPS. Door aan te tonen dat HGPS-cellulaire fenotypes omkeerbaar zijn, brengt deze studie wetenschappers een stap dichter bij het genezen van deze verwoestende kinderziekte.<\/p>\n<p>&quot;We wilden weten of deze cellulaire veranderingen die met Progeria gepaard gaan permanent zijn of teruggedraaid kunnen worden,&quot; aldus Scaffidi. De onderzoekers ontwierpen een &quot;moleculaire pleister\u00ae,&quot; aldus Misteli (een chemisch stabiele DNA-oligonucleotide, zodat de cel het niet kan afbreken.) Een week later was het gemuteerde lamin A-eiwit ge\u00eblimineerd en zagen meer dan 90 procent van de progeriacellen er normaal uit; en de activiteit van verschillende genen die verkeerd gereguleerd zijn bij HGPS-pati\u00ebnten, werd ook weer normaal. &quot;Het is verbazingwekkend dat we een zieke cel konden nemen en een paar dagen later was deze gezond en klaar om zich weer te delen,&quot; aldus Misteli.<\/p>\n<p>Hij merkte op dat deze resultaten het principe aantonen dat de cellulaire effecten van Progeria kunnen worden teruggedraaid. Dit betekent dat de methode van zijn laboratorium in de toekomst wellicht kan worden gebruikt als therapeutische strategie.<\/p>\n<p><strong>Een verlaagd niveau van \u2018goed\u2019 cholesterol bij kinderen met Progeria kan bijdragen aan vroegtijdige hartziekten.<\/strong> Adiponectine, een hormoon dat de stofwisseling van vet en suiker reguleert, kan nuttig zijn bij het vinden van behandelingen.<br \/><em>Het tijdschrift voor kindergeneeskunde, maart 2005<\/em><\/p>\n<p>Een team van onderzoekers onder leiding van de medisch directeur van PRF en assistent-professor Leslie Gordon, MD, PhD van de Tufts University School of Medicine, ontdekte dat kinderen met Progeria in hun midden- en latere jaren een verlaagd niveau van HDL-cholesterol hebben - of &quot;beschermend&quot; cholesterol - en adiponectine, een hormoon dat het metabolisme van vet en suiker reguleert. Beide factoren werken om vet van plaques in slagaders te verwijderen, en de lagere niveaus kunnen bijdragen aan versnelde plaquevorming. &quot;Alle kinderen met Progeria sterven tussen de leeftijd van 6 en 20 jaar aan hartfalen of een beroerte&quot;, aldus Dr. Gordon. &quot;Het bestuderen van hartziekten in relatie tot kinderen met Progeria kan ons helpen beter te begrijpen hoe atherosclerose de vergrijzende bevolking zal be\u00efnvloeden en tegelijkertijd deze kostbare kinderen zal helpen.&quot;<\/p>\n<p>&quot;Deze bevindingen zijn belangrijk voor de potenti\u00eble ontwikkeling van een behandeling voor Progeria&quot;, aldus de hoofdauteur van de studie<strong> Alice H. Lichtenstein, DSc, Human Nutrition Research Center on Aging aan de Tufts University. <\/strong>&quot;Als er betrouwbare medicijnen beschikbaar komen die worden gebruikt om de HDL-cholesterol- en adiponectinespiegels te verhogen, kunnen ze nuttig blijken bij het voorkomen van de progressie van atherosclerose bij kinderen met Progeria.&quot;<\/p>\n<p>\u201cDeze gegevens onderstrepen ook het belang van vetweefsel als actief weefsel dat hormonen afscheidt die de metabolische functie van het hele lichaam kunnen be\u00efnvloeden \u2013 een concept dat niet alleen belangrijk is voor Progeria, maar ook voor meer voorkomende ziekten zoals obesitas en diabetes\u201d, aldus de auteur. <strong>Mary Elizabeth Patti, MD, Joslin Diabetes Center en afdeling Geneeskunde, 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\/nl\/2005\/05\/16\/reversal-of-the-cellular-phenotype-in-the-premature-aging-disease-hgps\/\" \/>\n<meta property=\"og:locale\" content=\"nl_NL\" \/>\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\/nl\/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\":\"nl-NL\",\"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\/nl\/2005\/05\/16\/reversal-of-the-cellular-phenotype-in-the-premature-aging-disease-hgps\/","og_locale":"nl_NL","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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