{"id":93566,"date":"2024-02-28T14:26:00","date_gmt":"2024-02-28T13:26:00","guid":{"rendered":"https:\/\/port.lukasiewicz.gov.pl\/?p=93566"},"modified":"2025-12-23T11:03:00","modified_gmt":"2025-12-23T10:03:00","slug":"huntingtons-disease-at-the-center-of-interest-for-lukasiewicz-port-researchers","status":"publish","type":"post","link":"https:\/\/port.lukasiewicz.gov.pl\/en\/huntingtons-disease-at-the-center-of-interest-for-lukasiewicz-port-researchers\/","title":{"rendered":"Huntington\u2019s Disease at the Center of Interest for \u0141ukasiewicz \u2013 PORT Researchers"},"content":{"rendered":"\t\t
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Scientists at the Life Sciences and Biotechnology Center at \u0141ukasiewicz \u2013 PORT work every day on therapies that may contribute to breakthroughs in the treatment of severe disorders. On Rare Disease Day, observed annually on the last day of February, we present the latest approach to treating Huntington\u2019s disease, which is the subject of research conducted by Dr. Agnieszka Krzy\u017cosiak and the Neurodegeneration Mechanisms Research Group she leads at our Institute.<\/p>\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t

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Huntington\u2019s disease is a rare genetic neurodegenerative disorder that affects an estimated 5 in 100,000 people. As the disease progresses, its most severe symptoms include uncoordinated, involuntary movements, motor problems, emotional symptoms such as depression, and cognitive impairment.<\/p>

It is the most common neurodegenerative disease caused by a single genetic factor. The direct cause is the expansion of a polyglutamine chain in the huntingtin protein, leading to protein aggregation and, consequently, a series of cellular dysfunctions, deregulation of cellular proteostasis, and ultimately \u2013 cell death, meaning the death of neurons in the brain.<\/p>

One of the major risk factors is age (35\u201350 years).<\/p>

\u201cIn our work, we investigate the molecular basis of Huntington\u2019s disease, focusing primarily on protein quality control processes to better understand the accumulation of huntingtin in the cell,\u201d explains Dr. Agnieszka Krzy\u017cosiak, Leader of the Neurodegeneration Mechanisms Research Group at \u0141ukasiewicz \u2013 PORT. \u201cOur earlier studies confirmed that a cell in which proteostasis has been pharmacologically enhanced is better able to cope with the aggregation of disease-causing protein. Knowing that this approach \u2013 targeting protein quality control processes \u2013 is effective, we are currently searching for new therapeutic targets,\u201d adds Dr. Agnieszka Krzy\u017cosiak.<\/p>\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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Innovation in Research\n<\/h2>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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What makes Huntington\u2019s disease research at \u0141ukasiewicz \u2013 PORT innovative is the use of human patient-derived models as an alternative to animal models. The research method, based on reprogramming unaffected cells (e.g., skin fibroblasts) into neurons, was developed by Prof. Andrew Yoo (USA). The innovative nature of this method is demonstrated by its ability to reprogram cells in such a way that they reflect both the genetic and epigenetic background of the disease.<\/p>

With the latest tools and research methods, and scientists who have gained expertise in this disease at international research centers, we hope to move closer to establishing new standards in the treatment of Huntington\u2019s disease.<\/p>\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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Scientists at the Life Sciences and Biotechnology Center at \u0141ukasiewicz \u2013 PORT work every day on therapies that may contribute to breakthroughs in the treatment of severe disorders. On Rare Disease Day, observed annually on the last day of February, we present the latest approach to treating Huntington\u2019s disease, which is the subject of research conducted by Dr. Agnieszka Krzy\u017cosiak and the Neurodegeneration Mechanisms Research Group she leads at our Institute.<\/p>\n","protected":false},"author":218,"featured_media":96498,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"_seopress_robots_primary_cat":"none","_seopress_titles_title":"","_seopress_titles_desc":"","_seopress_robots_index":"","inline_featured_image":false,"footnotes":""},"categories":[204],"tags":[690],"class_list":["post-93566","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-uncategorized","tag-researches"],"acf":{"czas_czytania":"3","order":"0"},"publishpress_future_action":{"enabled":false,"date":"2026-04-24 12:57:19","action":"change-status","newStatus":"draft","terms":[],"taxonomy":"category","extraData":[]},"publishpress_future_workflow_manual_trigger":{"enabledWorkflows":[]},"_links":{"self":[{"href":"https:\/\/port.lukasiewicz.gov.pl\/en\/wp-json\/wp\/v2\/posts\/93566","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/port.lukasiewicz.gov.pl\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/port.lukasiewicz.gov.pl\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/port.lukasiewicz.gov.pl\/en\/wp-json\/wp\/v2\/users\/218"}],"replies":[{"embeddable":true,"href":"https:\/\/port.lukasiewicz.gov.pl\/en\/wp-json\/wp\/v2\/comments?post=93566"}],"version-history":[{"count":5,"href":"https:\/\/port.lukasiewicz.gov.pl\/en\/wp-json\/wp\/v2\/posts\/93566\/revisions"}],"predecessor-version":[{"id":95616,"href":"https:\/\/port.lukasiewicz.gov.pl\/en\/wp-json\/wp\/v2\/posts\/93566\/revisions\/95616"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/port.lukasiewicz.gov.pl\/en\/wp-json\/wp\/v2\/media\/96498"}],"wp:attachment":[{"href":"https:\/\/port.lukasiewicz.gov.pl\/en\/wp-json\/wp\/v2\/media?parent=93566"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/port.lukasiewicz.gov.pl\/en\/wp-json\/wp\/v2\/categories?post=93566"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/port.lukasiewicz.gov.pl\/en\/wp-json\/wp\/v2\/tags?post=93566"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}