日期:
2020 年 — 2026 年
2020
2021
2022
2023
2024
2025
2026
影响因子:

Accelerating Leigh syndrome drug discovery through deep learning screening in brain organoids

利用脑类器官深度学习筛选加速莱氏综合征药物研发

Menacho, Carmen; Okawa, Satoshi; Álvarez-Merz, Iris; Wittich, Annika; Muñoz-Oreja, Mikel; Lisowski, Pawel; Martín, Mario López; Pentimalli, Tancredi Massimo; Zakin, Shiri; Thevandavakkam, Mathuravani; Jerred, Caleb; Lickfett, Selene; Petersilie, Laura; Rybak-Wolf, Agnieszka; Seibt, Annette; Herebian, Diran; Inak, Gizem; Brodesser, Susanne; Zaliani, Andrea; Mlody, Barbara; Donnelly, Justin; Woleben, Kasey; Soriano, Francesc Xavier; Fernandez-Checa, Jose C; Ventura, Natascia; Cambridge, Sidney; Mayatepek, Ertan; Spinazzola, Antonella; Schuelke, Markus; Rajewsky, Nikolaus; Rossi, Andrea; Peralvarez-Marin, Alex; Distelmaier, Felix; Perlstein, Ethan; Holt, Ian J; Puighermanal, Emma; Pless, Ole; Rose, Christine R; Del Sol, Antonio; Prigione, Alessandro

Mutant huntingtin impairs neurodevelopment in human brain organoids through CHCHD2-mediated neurometabolic failure

突变型亨廷顿蛋白通过CHCHD2介导的神经代谢障碍损害人脑类器官的神经发育。

Pawel Lisowski # ,Selene Lickfett # ,Agnieszka Rybak-Wolf ,Carmen Menacho ,Stephanie Le ,Tancredi Massimo Pentimalli ,Sofia Notopoulou ,Werner Dykstra ,Daniel Oehler ,Sandra López-Calcerrada ,Barbara Mlody ,Maximilian Otto ,Haijia Wu ,Yasmin Richter ,Philipp Roth ,Ruchika Anand ,Linda A M Kulka ,David Meierhofer ,Petar Glazar ,Ivano Legnini ,Narasimha Swamy Telugu ,Tobias Hahn ,Nancy Neuendorf ,Duncan C Miller ,Annett Böddrich ,Amin Polzin ,Ertan Mayatepek ,Sebastian Diecke ,Heidi Olzscha ,Janine Kirstein ,Cristina Ugalde ,Spyros Petrakis ,Sidney Cambridge ,Nikolaus Rajewsky ,Ralf Kühn ,Erich E Wanker ,Josef Priller ,Jakob J Metzger ,Alessandro Prigione

Generation of four iPSC lines from four patients with Leigh syndrome carrying homoplasmic mutations m.8993T > G or m.8993T > C in the mitochondrial gene MT-ATP6

从四名携带线粒体基因 MT-ATP6 同质性突变 m.8993T > G 或 m.8993T > C 的 Leigh 综合征患者体内生成四株 iPSC 细胞系

Carmen Lorenz, Annika Zink, Marie-Therese Henke, Selma Staege, Barbara Mlody, Miriam Bünning, Erich Wanker, Sebastian Diecke, Markus Schuelke, Alessandro Prigione

Generation of induced pluripotent stem cells from three individuals with Huntington's disease

从三名亨廷顿氏病患者体内产生诱导性多能干细胞

Duncan C Miller, Pawel Lisowski, Selene Lickfett, Barbara Mlody, Miriam Bünning, Carolin Genehr, Claas Ulrich, Erich E Wanker, Sebastian Diecke, Josef Priller, Alessandro Prigione

Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome

代谢编程缺陷会损害神经培养物和莱氏综合征类器官模型中的早期神经元形态发生。

Gizem Inak ,Agnieszka Rybak-Wolf ,Pawel Lisowski ,Tancredi M Pentimalli ,René Jüttner ,Petar Glažar ,Karan Uppal ,Emanuela Bottani ,Dario Brunetti ,Christopher Secker ,Annika Zink ,David Meierhofer ,Marie-Thérèse Henke ,Monishita Dey ,Ummi Ciptasari ,Barbara Mlody ,Tobias Hahn ,Maria Berruezo-Llacuna ,Nikos Karaiskos ,Michela Di Virgilio ,Johannes A Mayr ,Saskia B Wortmann ,Josef Priller ,Michael Gotthardt ,Dean P Jones ,Ertan Mayatepek ,Werner Stenzel ,Sebastian Diecke ,Ralf Kühn ,Erich E Wanker ,Nikolaus Rajewsky ,Markus Schuelke ,Alessandro Prigione

Complete suppression of Htt fibrillization and disaggregation of Htt fibrils by a trimeric chaperone complex

三聚体分子伴侣复合物完全抑制Htt纤维化并解聚Htt纤维。

Scior, Annika; Arnsburg, Kristin; Iburg, Manuel; Juenemann, Katrin; Lucia Pigazzini, Maria; Mlody, Barbara; Puchkov, Dmytro; Ast, Anne; Buntru, Alexander; Priller, Josef; Wanker, Erich E; Prigione, Alessandro; Kirstein, Janine

Complete suppression of Htt fibrilization and disaggregation of Htt fibrils by a trimeric chaperone complex

三聚体分子伴侣复合物完全抑制Htt纤维化并解聚Htt纤维。

Annika Scior ,Alexander Buntru ,Kristin Arnsburg ,Anne Ast ,Manuel Iburg ,Katrin Juenemann ,Maria Lucia Pigazzini ,Barbara Mlody ,Dmytro Puchkov ,Josef Priller ,Erich E Wanker ,Alessandro Prigione ,Janine Kirstein

Mitochondria and the dynamic control of stem cell homeostasis

线粒体与干细胞稳态的动态调控

Lisowski, Pawel; Kannan, Preethi; Mlody, Barbara; Prigione, Alessandro

Human iPSC-Derived Neural Progenitors Are an Effective Drug Discovery Model for Neurological mtDNA Disorders

人类 iPSC 衍生的神经祖细胞是治疗神经系统线粒体 DNA 疾病的有效药物发现模型

Carmen Lorenz, Pierre Lesimple, Raul Bukowiecki, Annika Zink, Gizem Inak, Barbara Mlody, Manvendra Singh, Marcus Semtner, Nancy Mah, Karine Auré, Megan Leong, Oleksandr Zabiegalov, Ekaterini-Maria Lyras, Vanessa Pfiffer, Beatrix Fauler, Jenny Eichhorst, Burkhard Wiesner, Norbert Huebner, Josef Prill

Nijmegen Breakage Syndrome fibroblasts and iPSCs: cellular models for uncovering disease-associated signaling pathways and establishing a screening platform for anti-oxidants

奈梅亨断裂综合征成纤维细胞和 iPSC:揭示疾病相关信号通路和建立抗氧化剂筛选平台的细胞模型

Barbara Mlody, Wasco Wruck, Soraia Martins, Karl Sperling, James Adjaye