Results 221 to 230 of about 945,929 (245)

NARFL Knockout Triggers Ferroptosis‐Driven Vascular Endothelial Dysfunction

Advanced Science, EarlyView.
NARFL is vital for CIA and oxidative stress resistance. NARFL deletion in HPMEC cells, zebrafish, and mice is lethal and rescued by a Ferroptosis inhibitor. NARFL deficiency disrupted its interaction with CIA proteins, decreased aconitase activity, increased IRP1 activity, induced Fe overload, and led to ferroptosis and oxidative stress, resulting in ...
Hui Hu, Jing Luo, Li Yu, Daoxi Qi, Boyu Li, Yating Chen, Chen Wang, Xiaokang Zhang, Wenzheng Guo, Qiyong Lou, Gang Zhai, Yonglin Ruan, Jianfei Huang, Shengchi Shi, Zhan Yin, Fang Zheng   +15 more
wiley   +1 more source

Small molecules restore mutant mitochondrial DNA polymerase activity. [PDF]

Nature
Valenzuela S, Zhu X, Macao B, Stamgren M, Geukens C, Charifson PS, Kern G, Hoberg E, Jenninger L, Gruszczyk AV, Lee S, Johansson KAS, Miralles Fusté J, Shi Y, Kerns SJ, Arabanian L, Martinez Botella G, Ekström S, Green J, Griffin AM, Pardo-Hernández C, Keating TA, Küppers-Munther B, Larsson NG, Phan C, Posse V, Jones JE, Xie X, Giroux S, Gustafsson CM, Falkenberg M.   +30 more
europepmc   +1 more source

RIG‐I Mediated Neuron‐Specific IFN Type 1 Signaling in FUS‐ALS Induces Neurodegeneration and Offers New Biomarker‐Driven Individualized Treatment Options for (FUS‐)ALS

Advanced Science, EarlyView.
Using iPSC‐derived motoneurons and postmortem tissue from FUS‐ALS patients, it is demonstrated that increased mitochondrial transcription leads to elevated cytosolic double‐stranded RNA (dsRNA) levels. This aberrant accumulation activates a RIG‐I–dependent innate immune response leading to neurodegeneration, which is amenable for FDA‐ and EMA‐approved ...
Marcel Naumann, Theresa M. Wierschin, Stefanie Kretschmer, Banaja P. Dash, Aaron Held, Andrea Salzinger, Kevin Peikert, Anže Karlek, Hannes Glaß, Dajana Großmann, René Günther, Susanne Petri, Annekathrin Rödiger, David Brenner, Francisco Pan‐Montojo, Eleonora Aronica, Markus Kipp, Vitaly Zimyanin, Jared Sterneckert, Torsten Grehl, Noah D. Seebacher, Tobias M. Böckers, Alberto Catanese, Brian J. Wainger, Patrick Oeckl, Min Ae Lee‐Kirsch, Andreas Hermann   +26 more
wiley   +1 more source

Allosteric Modulation of Pathological Ataxin‐3 Aggregation: A Path to Spinocerebellar Ataxia Type‐3 Therapies

Advanced Science, EarlyView.
This study uncovers a new allosteric site in the Josephin domain of ataxin‐3 targeted by the molecular tweezer CLR01, which modulates protein aggregation, improves synaptic function in neuronal cells, and delays motor dysfunction in animal models.
Alexandra Silva, Sara Duarte‐Silva, Pedro M. Martins, Beatriz Rodrigues, Débora Serrenho, Daniela Vilasboas‐Campos, Andreia Teixeira‐Castro, Julio Vieyto‐Nuñez, Joel Mieres‐Perez, Francisco Figueiredo, Martyna Podlasiak, Tatiana Van‐der‐Kellen, Joana Fraga, James Noble, Carter Lantz, Niki Sepanj, Daniela Monteiro‐Fernandes, Sara Guerreiro, Andreia Neves‐Carvalho, Joana Pereira‐Sousa, Frank‐Gerrit Klärner, Thomas Schrader, Joseph A. Loo, Annalisa Pastore, Elsa Sanchez‐Garcia, Gal Bitan, Ana Luísa Carvalho, Patrícia Maciel, Sandra Macedo‐Ribeiro   +28 more
wiley   +1 more source

Precise mapping of single-stranded DNA breaks by sequence-templated erroneous DNA polymerase end-labelling. [PDF]

Nat Commun
Wenson L, Heldin J, Martin M, Erbilgin Y, Salman B, Sundqvist A, Schaal W, Sandbaumhüter FA, Jansson ET, Chen X, Davidsson A, Stenerlöw B, Espinoza JA, Lindström M, Lennartsson J, Spjuth O, Söderberg O.   +16 more
europepmc   +1 more source

A Patient‐Derived Organoid Biobank of Adamantinomatous Craniopharyngioma as a Platform for Drug Discovery

Advanced Science, EarlyView.
This study successfully establishes adamantinomatous craniopharyngioma (ACP) patient‐derived organoids (PDOs) that preserve the histopathological and genetic features of the original tumors. Through drug sensitivity assays and subsequent mechanistic analyses, the study demonstrates that Ceritinib exerts its inhibitory effects on ACP PDO growth by ...
Huarong Zhang, Chaohu Wang, Jun Fan, Zexin Chen, Haoying Yu, Yawen Bai, Tingcheng Zhang, Qianchao Zhu, Yiwen Feng, Peirong Niu, Jiaqian Chen, Liping Yang, Xueying Li, Lei Yu, Songtao Qi, Yi Liu   +15 more
wiley   +1 more source

Engineering of novel DNA polymerase variants for single enzyme quantitative multiplex reverse transcription-PCR. [PDF]

Sci Rep
Huber LB, Marchand V, Somtürk M, Müller S, Marx A.   +4 more
europepmc   +1 more source

CCDC41 Drives Oocyte Meiotic Progression by Promoting Rab11a/Rab7‐Positive Vesicle Fusion with Target Membranes

Advanced Science, EarlyView.
CCDC41 is essential for meiotic maturation in mouse oocytes through regulating Rab7‐positive endosomes fusion with lysosomes and Rab11a‐positive vesicle fusion with the plasma membrane. Abstract Coiled‐coil domain‐containing protein 41 (CCDC41), a core component of centriolar distal appendages involved in centriole assembly and ciliary vesicle docking,
Ying Tian, Jiatong Li, Jingyi Kang, Xiangning Xu, Bicheng Wang, Shuo Lou, Jingyu Li, Yuying Yang, Yanbing Zhang, Yangzi Zheng, Jing Weng, Yuanjing Liang, Wei Ma   +12 more
wiley   +1 more source

Structural basis of the phosphoramidate N-benzimidazole group's influence on modified primer extension efficiency by Taq DNA polymerase. [PDF]

Vavilovskii Zhurnal Genet Selektsii
Berdugin AA, Golyshev VM, Lomzov AA.
europepmc   +1 more source