{"id":7482,"date":"2026-01-06T19:14:31","date_gmt":"2026-01-06T19:14:31","guid":{"rendered":"https:\/\/cureclcn4.org\/cure-clcn4-gefoerderte-projekte\/"},"modified":"2026-01-06T19:14:32","modified_gmt":"2026-01-06T19:14:32","slug":"cure-clcn4-gefoerderte-projekte","status":"publish","type":"page","link":"https:\/\/cureclcn4.org\/de\/cure-clcn4-gefoerderte-projekte\/","title":{"rendered":"Cure CLCN4-gef\u00f6rderte Projekte"},"content":{"rendered":"

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Im Folgenden finden Sie eine Liste von Forschungsinitiativen, die wir mit Stolz unterst\u00fctzt haben. Diese Projekte stehen im Mittelpunkt unserer Mission, die darin besteht, eine Zusammenarbeit mit engagierten Forschern und Klinikern aufzubauen, um die Biologie der CLCN4-bedingten<\/em> Erkrankung zu entschl\u00fcsseln und die Lebensqualit\u00e4t der Betroffenen zu verbessern. <\/span><\/h1>\n

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Gemeinsam treiben wir den Fortschritt Schritt f\u00fcr Schritt voran<\/h2>\n

[\/et_pb_text][\/et_pb_column][\/et_pb_row][et_pb_row custom_padding_last_edited=“on|phone“ _builder_version=“4.16″ background_color=“#ffffff“ max_width=“1200px“ custom_padding=“29px|80px|28px|80px|false|false“ custom_padding_tablet=“|80px||80px|false|true“ custom_padding_phone=“40px|30px||30px||true“ box_shadow_style=“preset3″ box_shadow_vertical=“40px“ box_shadow_blur=“100px“ box_shadow_spread=“-30px“ box_shadow_color=“rgba(0,0,0,0.22)“ use_custom_width=“on“ custom_width_px=“1200px“ global_colors_info=“{}“][et_pb_column type=“4_4″ _builder_version=“4.16″ custom_padding=“|||“ global_colors_info=“{}“ custom_padding__hover=“|||“][et_pb_blurb title=“Cure clcn4 Pilot-Zuschussprogramm 2023 – Bekanntgabe der Preistr\u00e4ger im MAI 2024″ content_max_width=“800px“ _builder_version=“4.23.1″ header_font=“|700||on|||||“ header_font_size=“18px“ header_line_height=“2em“ body_font_size=“17px“ body_line_height=“2em“ global_colors_info=“{}“]<\/p>\n

Bewerbungen f\u00fcr das Cure CLCN4 Pilot Grant Programm 2023 sind offen (Frist 15. Dezember 2023)! Mit dem Zuschuss werden insgesamt 150.000 \u00a3 f\u00fcr 2-3 Forschungsprojekte zur Verf\u00fcgung gestellt, die sich auf CLCN4-bezogene<\/em> Erkrankungen konzentrieren (50.000-75.000 \u00a3\/jedes Projekt). Einzelheiten zu den ausgezeichneten Projekten werden hier Anfang Mai bekannt gegeben. F\u00fcr weitere Informationen \u00fcber den Zuschuss klicken Sie bitte hier<\/a>. <\/strong><\/p>\n

[\/et_pb_blurb][\/et_pb_column][\/et_pb_row][et_pb_row custom_padding_last_edited=“on|phone“ _builder_version=“4.16″ background_color=“#ffffff“ max_width=“1200px“ custom_padding=“29px|80px|28px|80px|false|false“ custom_padding_tablet=“|80px||80px|false|true“ custom_padding_phone=“40px|30px||30px||true“ box_shadow_style=“preset3″ box_shadow_vertical=“40px“ box_shadow_blur=“100px“ box_shadow_spread=“-30px“ box_shadow_color=“rgba(0,0,0,0.22)“ use_custom_width=“on“ custom_width_px=“1200px“ global_colors_info=“{}“][et_pb_column type=“4_4″ _builder_version=“4.16″ custom_padding=“|||“ global_colors_info=“{}“ custom_padding__hover=“|||“][et_pb_blurb title=“%912023 – present%93 – Herstellung eines Anti-CIC-4-Antik\u00f6rpers zur Verwendung in der Grundlagenforschung“ content_max_width=“800px“ _builder_version=“4.23.1″ header_font=“|700||on|||||“ header_font_size=“18px“ header_line_height=“2em“ body_font_size=“17px“ body_line_height=“2em“ global_colors_info=“{}“]<\/p>\n

Grundprinzipien:<\/strong> Antik\u00f6rper sind wichtige Werkzeuge in der Grundlagenforschung, die den spezifischen Nachweis von Proteinen von Interesse erm\u00f6glichen. Diese Nachweism\u00f6glichkeit ist f\u00fcr das Verst\u00e4ndnis des Expressionsniveaus von Proteinen, ihrer Ver\u00e4nderungen und ihrer Lokalisierung in Zellen oder Geweben unerl\u00e4sslich. Durch den Einsatz von Antik\u00f6rpern k\u00f6nnen Forscher kartieren, wo und wann ein Protein exprimiert wird, beobachten, wie sich seine Expression unter verschiedenen Bedingungen ver\u00e4ndert, und Einblicke in seine Rolle bei verschiedenen zellul\u00e4ren Prozessen gewinnen. Derzeit gibt es keine unabh\u00e4ngig validierten, allgemein verf\u00fcgbaren Antik\u00f6rper f\u00fcr den Nachweis von CIC-4, dem vom CLCN4-Gen<\/em> kodierten Protein, was ein Hindernis f\u00fcr die Grundlagenforschung darstellt. <\/p>\n

Ziel:<\/strong> Das Ziel dieses Projekts ist die Entwicklung einer Reihe von funktionierenden Antik\u00f6rpern f\u00fcr den spezifischen Nachweis des CIC-4-Proteins. Projekte zur Herstellung eines polyklonalen und eines monoklonalen Antik\u00f6rpers sind im Gange (weitere Details folgen in K\u00fcrze!). <\/p>\n

Ergebnis (in Arbeit)<\/strong><\/p>\n

[\/et_pb_blurb][\/et_pb_column][\/et_pb_row][et_pb_row custom_padding_last_edited=“on|phone“ _builder_version=“4.16″ background_color=“#ffffff“ max_width=“1200px“ custom_padding=“29px|80px|28px|80px|false|false“ custom_padding_tablet=“|80px||80px|false|true“ custom_padding_phone=“40px|30px||30px||true“ box_shadow_style=“preset3″ box_shadow_vertical=“40px“ box_shadow_blur=“100px“ box_shadow_spread=“-30px“ box_shadow_color=“rgba(0,0,0,0.22)“ use_custom_width=“on“ custom_width_px=“1200px“ global_colors_info=“{}“][et_pb_column type=“4_4″ _builder_version=“4.16″ custom_padding=“|||“ global_colors_info=“{}“ custom_padding__hover=“|||“][et_pb_blurb title=“%912021 – 2023%93 – Projekt zur Erstellung von Clcn4-Rattenmodellen (\u00a350.000)“ content_max_width=“800px“ _builder_version=“4.23.1″ header_font=“|700||on|||||“ header_font_size=“17px“ header_line_height=“2em“ body_font_size=“17px“ body_line_height=“2em“ global_colors_info=“{}“]<\/p>\n

Grundprinzipien:<\/strong> Die Erforschung der CLCN4-bedingten<\/em> neurologischen Entwicklungsst\u00f6rung(CLCN4-NDC<\/em>) wurde durch das Fehlen eines geeigneten Tiermodells behindert. Fr\u00fcher erzeugte Clcn4-Knockout<\/em> (KO)-Mauslinien zeigten keinen offensichtlichen Ph\u00e4notyp (normale Entwicklung, keine anatomischen Ver\u00e4nderungen im Gehirn und keine offensichtlichen Verhaltensanomalien)[1<\/a>,2<\/a>,3<\/a>]. Dies k\u00f6nnte daran liegen, dass das Clcn4-Gen<\/em> der Maus autosomal und nicht X-chromosomal vererbt wird. Mit dem Ziel, diese H\u00fcrde zu \u00fcberwinden und in Anbetracht der Tatsache, dass das Clcn4-Gen<\/em> bei Ratten auf dem X-Chromosom liegt, was bedeutet, dass das Clcn4-Gen<\/em> der Ratte selbst und seine Umgebung im Genom besser konserviert sind, hat Cure CLCN4 versucht, ein Rattenmodell f\u00fcr CLCN4-NDC<\/em> zu entwickeln. <\/p>\n

Ziel:<\/strong> Ziel dieses Projekts war es, drei Clcn4-Rattenmodelle<\/em> zu generieren: i) eine Clcn4-Knockout-Ratte<\/em>, ii) eine Clcn4-Konditions-Knockout-Ratte<\/em> und iii) eine Clcn4-Knock-in<\/em> (KI)-Linie, die das Ratten\u00e4quivalent der p.(Ala555Val) CLCN4-Variante<\/em> tr\u00e4gt, der am h\u00e4ufigsten wiederkehrenden Variante mit einem starken Funktionsgewinn-Ph\u00e4notyp im Xenopus-Modell<\/em>.<\/p>\n

Das Ergebnis:<\/strong> Mit finanzieller Unterst\u00fctzung von Cure CLCN4 und durchgef\u00fchrt vom IGBMC (Institut de g\u00e9n\u00e9tique et de biologie mol\u00e9culaire et cellulaire, Frankreich) wurde dieses Projekt erfolgreich abgeschlossen und alle drei Rattenmodelle sind auf dem INFRAFRONTIER-Repository verf\u00fcgbar. F\u00fcr weitere Informationen und Bestellm\u00f6glichkeiten senden Sie uns bitte eine E-Mail an info@cureclcn4.org <\/p>\n

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Nehmen Sie Kontakt auf. Engagieren Sie sich.<\/h2>\n

[\/et_pb_text][\/et_pb_column][\/et_pb_row][et_pb_row _builder_version=“4.16″ background_color=“#ffffff“ max_width=“850px“ custom_padding=“60px|60px|60px|60px|true|true“ animation_style=“slide“ animation_direction=“top“ animation_intensity_slide=“2%“ box_shadow_style=“preset3″ box_shadow_vertical=“40px“ box_shadow_blur=“100px“ box_shadow_spread=“-30px“ box_shadow_color=“rgba(0,0,0,0.22)“ use_custom_width=“on“ custom_width_px=“850px“ global_colors_info=“{}“][et_pb_column type=“4_4″ _builder_version=“4.16″ custom_padding=“|||“ global_colors_info=“{}“ custom_padding__hover=“|||“][et_pb_contact_form email=“info@cureclcn4.org“ success_message=“Vielen Dank f\u00fcr die Kontaktaufnahme. 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url=“https:\/\/uk.linkedin.com\/company\/cure-clcn4?trk=public_profile_experience-item_result-card_image-click“ _builder_version=“4.16″ _module_preset=“default“ background_color=“rgba(255,255,255,0.2)“ custom_margin=“|6px||6px|false|false“ custom_padding=“8px|8px|8px|8px|false|false“ global_colors_info=“{}“ follow_button=“off“ url_new_window=“on“]linkedin[\/et_pb_social_media_follow_network][\/et_pb_social_media_follow][\/et_pb_column][\/et_pb_row][\/et_pb_section]<\/p>\n","protected":false},"excerpt":{"rendered":"

Hier finden Sie eine Liste von Forschungsprojekten, die wir mit Stolz unterst\u00fctzt haben. Diese Projekte stehen im Mittelpunkt unserer Mission, die darin besteht, eine Zusammenarbeit mit engagierten Forschern und Klinikern aufzubauen, um die Biologie der CLCN4-Krankheit zu entschl\u00fcsseln und die Lebensqualit\u00e4t der Betroffenen zu verbessern. <\/p>\n","protected":false},"author":2,"featured_media":4778,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_et_pb_use_builder":"on","_et_pb_old_content":"","_et_gb_content_width":"","footnotes":""},"class_list":["post-7482","page","type-page","status-publish","has-post-thumbnail","hentry"],"_links":{"self":[{"href":"https:\/\/cureclcn4.org\/de\/wp-json\/wp\/v2\/pages\/7482","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/cureclcn4.org\/de\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/cureclcn4.org\/de\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/cureclcn4.org\/de\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/cureclcn4.org\/de\/wp-json\/wp\/v2\/comments?post=7482"}],"version-history":[{"count":2,"href":"https:\/\/cureclcn4.org\/de\/wp-json\/wp\/v2\/pages\/7482\/revisions"}],"predecessor-version":[{"id":7484,"href":"https:\/\/cureclcn4.org\/de\/wp-json\/wp\/v2\/pages\/7482\/revisions\/7484"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/cureclcn4.org\/de\/wp-json\/wp\/v2\/media\/4778"}],"wp:attachment":[{"href":"https:\/\/cureclcn4.org\/de\/wp-json\/wp\/v2\/media?parent=7482"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}