{"id":26814,"date":"2010-08-03T18:00:00","date_gmt":"2010-08-03T16:00:00","guid":{"rendered":"https:\/\/www.embl.org\/news\/?p=26814"},"modified":"2024-11-14T16:31:21","modified_gmt":"2024-11-14T15:31:21","slug":"supply-and-demand","status":"publish","type":"post","link":"https:\/\/www.embl.org\/news\/science\/supply-and-demand\/","title":{"rendered":"Supply and demand"},"content":{"rendered":"\n<div class=\"wp-block-image\"><figure class=\"vf-figure  | vf-figure--align vf-figure--align-inline-start   size-large is-resized\"><a href=\"https:\/\/www.embl.org\/news\/?attachment_id=26838\"><img loading=\"lazy\" decoding=\"async\" class=\"vf-figure__image\" src=\"https:\/\/www.embl.org\/news\/wp-content\/uploads\/2020\/05\/mitochondria-em-389x1024.jpg\" alt=\"These electron microscopy images show mitochondria in a normal cell (top) and a close-up of a mitochondrion with structural defects, in a cell that cannot produce IRPs (bottom).\" class=\"wp-image-26838\" width=\"195\" height=\"512\" srcset=\"https:\/\/www.embl.org\/news\/wp-content\/uploads\/2020\/05\/mitochondria-em-389x1024.jpg 389w, https:\/\/www.embl.org\/news\/wp-content\/uploads\/2020\/05\/mitochondria-em-114x300.jpg 114w, https:\/\/www.embl.org\/news\/wp-content\/uploads\/2020\/05\/mitochondria-em.jpg 624w\" sizes=\"auto, (max-width: 195px) 100vw, 195px\" \/><\/a><figcaption class=\"vf-figure__caption\">These electron microscopy images show mitochondria in a normal cell (top) and a close-up of a mitochondrion with structural defects, in a cell that cannot produce IRPs (bottom).<br \/>Image credits: Bruno Galy\/ EMBL<\/figcaption><\/figure><\/div>\n\n\n\n<p>Most organisms need iron to survive, but too much iron is toxic, and can cause fatal organ failure. The same is true inside cells, where iron balance must also be maintained. In a study published today in <em>Cell Metabolism<\/em>, scientists at the European Molecular Biology Laboratory (EMBL) in Heidelberg, Germany, have discovered that a group of proteins named IRPs ensure that this iron balance is kept and as such are essential for cell survival. More specifically, they found that IRPs are required for the functioning of mitochondria, the cell\u2019s energy factories.<\/p>\n\n\n\n<p>Mitochondria need iron in order to function, but they also convert iron into other chemical forms used throughout the cell: iron sulphur clusters and haem \u2013 one of the building blocks of haemoglobin. Thanks to new mouse models they engineered, the EMBL scientists have been able to selectively shut down IRP function in specific cell types such as hepatocytes, liver cells that carry out multiple vital metabolic functions.<\/p>\n\n\n\n<p>\u201cMice whose liver cells can\u2019t produce IRPs die of liver failure a few days after birth,\u201d says Bruno Galy, Staff Scientist in Matthias Hentze\u2019s group at EMBL, who spearheaded the work: \u201cThe mitochondria in those cells have structural defects and don\u2019t function properly, because they don\u2019t have enough iron.\u201d<\/p>\n\n\n\n<p>Galy and colleagues found that in cells that cannot produce IRPs, the mechanisms for iron export and storage go into over-drive, while iron import is drastically reduced. This combination of factors leads to an iron shortage in the cell. As a consequence, the mitochondria don\u2019t receive enough iron, so they can\u2019t function properly, and can\u2019t make enough haem and iron sulphur clusters available to the cell machinery that depends on them. In short, the role of IRPs is to ensure that there is enough iron available in the cell to sustain mitochondrial iron needs.<\/p>\n\n\n\n<p>\u201cWe have indications that this is probably a general process by which most cells control their iron content and secure mitochondrial iron sufficiency\u201d <a href=\"https:\/\/www.embl.org\/groups\/hentze\/\">Hentze<\/a> concludes.<\/p>\n\n\n\n<p>This mechanism for regulating iron balance could be particularly important in cells with very high mitochondrial iron needs, such as red blood cell precursors that manufacture copious amounts of haem for oxygen transport. However, this may well be a double-edged sword. Indeed, there are situations in which mitochondria get iron but are not able to make use of it. The cell interprets this as a sign of mitochondrial iron insufficiency and responds by activating IRPs, which ultimately results in detrimental iron overloading of mitochondria. This may underlie the pathology of several diseases including inherited sideroblastic anaemias \u2013 in which cells are unable to incorporate iron into haemoglobin \u2013 or the neurodegenerative disorder Friedreich\u2019s ataxia, which the EMBL scientists are currently investigating.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>Most organisms need iron to survive, but too much iron is toxic, and can cause fatal organ failure. The same is true inside cells, where iron balance must also be maintained. In a study published today in Cell Metabolism, scientists at the European Molecular Biology Laboratory (EMBL) in Heidelberg,&hellip;<\/p>\n","protected":false},"author":16,"featured_media":26838,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"footnotes":""},"categories":[2,17591],"tags":[406,1718,1748],"embl_taxonomy":[19285],"class_list":["post-26814","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-science","category-science-technology","tag-hentze","tag-mitochondria","tag-press-release","embl_taxonomy-hentze-group"],"acf":{"show_featured_image":true,"vf_locked":false,"featured":false,"article_intro":"<p>EMBL scientists identify proteins that ensure iron balance<\/p>\n","article_sources":[{"source_description":"<p>Galy, B., Ferring-Appel, D.,  Sauer, S. W.,  Kaden, S., Lyoumi, S., Puy, H., K\u00f6lker, S., Gr\u00f6ne, H. J., &#038; Hentze, M. W., Iron Regulatory Proteins Secure Mitochondrial Iron Sufficiency and Function, <em>Cell Metabolism<\/em>, 4 August 2010. DOI: 10.1016\/j.cmet.2010.06.007 <\/p>\n","source_link_url":"https:\/\/www.sciencedirect.com\/science\/article\/pii\/S1550413110002305?via%3Dihub"}],"related_links":false,"in_this_article":false,"color":"#007B53","youtube_url":"","mp4_url":"","video_caption":"","press_contact":"EMBL Generic","field_target_display":"embl","source_article":false},"embl_taxonomy_terms":[{"uuid":"a:3:{i:0;s:36:\"302cfdf7-365b-462a-be65-82c7b783ebf7\";i:1;s:36:\"6c3125e7-cd51-4991-af2d-d5f97e57a845\";i:2;s:36:\"645cb0c7-a45b-4317-a833-05bb8ea7f79a\";}","parents":[],"name":["Hentze Group"],"slug":"hentze-group","description":"What &gt; Directors' research &gt; Hentze Group"}],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v26.2 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Supply and demand | EMBL<\/title>\n<meta name=\"description\" content=\"EMBL scientists discovered that a group of proteins named IRPs ensure that iron balance is kept and as such are essential for cell survival.\" \/>\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.embl.org\/news\/science\/supply-and-demand\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Supply and demand | EMBL\" \/>\n<meta property=\"og:description\" content=\"EMBL scientists discovered that a group of proteins named IRPs ensure that iron balance is kept and as such are essential for cell survival.\" \/>\n<meta property=\"og:url\" content=\"https:\/\/www.embl.org\/news\/science\/supply-and-demand\/\" \/>\n<meta property=\"og:site_name\" content=\"EMBL\" \/>\n<meta property=\"article:publisher\" content=\"https:\/\/www.facebook.com\/embl.org\/\" \/>\n<meta property=\"article:published_time\" content=\"2010-08-03T16:00:00+00:00\" \/>\n<meta property=\"article:modified_time\" content=\"2024-11-14T15:31:21+00:00\" \/>\n<meta property=\"og:image\" content=\"https:\/\/www.embl.org\/news\/wp-content\/uploads\/2020\/05\/mitochondria-em.jpg\" \/>\n\t<meta property=\"og:image:width\" content=\"624\" \/>\n\t<meta property=\"og:image:height\" content=\"1642\" \/>\n\t<meta property=\"og:image:type\" content=\"image\/jpeg\" \/>\n<meta name=\"author\" content=\"Guest author(s)\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:creator\" content=\"@embl\" \/>\n<meta name=\"twitter:site\" content=\"@embl\" \/>\n<meta name=\"twitter:label1\" content=\"Written by\" \/>\n\t<meta name=\"twitter:data1\" content=\"Guest author(s)\" \/>\n\t<meta name=\"twitter:label2\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data2\" content=\"3 minutes\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\/\/schema.org\",\"@graph\":[{\"@type\":\"NewsArticle\",\"@id\":\"https:\/\/www.embl.org\/news\/science\/supply-and-demand\/#article\",\"isPartOf\":{\"@id\":\"https:\/\/www.embl.org\/news\/science\/supply-and-demand\/\"},\"author\":{\"name\":\"Guest author(s)\",\"@id\":\"https:\/\/www.embl.org\/news\/#\/schema\/person\/b4d9366b2ebe691c4015c64c3619205b\"},\"headline\":\"Supply and demand\",\"datePublished\":\"2010-08-03T16:00:00+00:00\",\"dateModified\":\"2024-11-14T15:31:21+00:00\",\"mainEntityOfPage\":{\"@id\":\"https:\/\/www.embl.org\/news\/science\/supply-and-demand\/\"},\"wordCount\":490,\"publisher\":{\"@id\":\"https:\/\/www.embl.org\/news\/#organization\"},\"image\":{\"@id\":\"https:\/\/www.embl.org\/news\/science\/supply-and-demand\/#primaryimage\"},\"thumbnailUrl\":\"https:\/\/www.embl.org\/news\/wp-content\/uploads\/2020\/05\/mitochondria-em.jpg\",\"keywords\":[\"hentze\",\"mitochondria\",\"press release\"],\"articleSection\":[\"Science\",\"Science &amp; 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