Results 51 to 60 of about 21,042 (237)

Light and Shadows in Newborn Screening for Lysosomal Storage Disorders: Eight Years of Experience in Northeast Italy [PDF]

, 2023
Vincenza Gragnaniello, Chiara Cazzorla, Daniela Gueraldi, Andrea Puma, Christian Loro, Elena Porcù, Maria Stornaiuolo, Paolo Miglioranza, Leonardo Salviati, Alessandro P. Burlina, Alberto Burlina   +10 more
openalex   +1 more source

Lnc‐HZ05 Suppresses Trophoblast Cell Migrasome Formation by Disrupting TGFβ2 Pathway in Unexplained Recurrent Miscarriage

Advanced Science, EarlyView.
TGFβ2 signaling‐mediated migration/invasion and migrasome formation are suppressed in recurrent miscarriage (RM) versus healthy control villous tissues and are negatively associated with unexplained RM. In mechanism, TGFβ2 promotes trophoblast cell migration/invasion and migrasome formation, all of which are suppressed by lnc‐HZ05. In details, lnc‐HZ05
Weina Chen, Ying Zhang, Zimeng Zhao, Yang Yang, Geng Guo, Yi Sun, Tao Zhang, Zhihong Zhang, Liqin Zeng, Wenxin Huang, Qingjun Shen, Ruining Han, Zhaohui He, Depeng Zhao, Huidong Zhang   +14 more
wiley   +1 more source

Navigating Transition Metal‐Dependent Cell Death: Mechanisms, Crosstalk, and Future Directions

Advanced Science, EarlyView.
Transition metals trigger distinct regulated cell death pathways beyond traditional apoptosis. This review examines ferroptosis (iron‐catalyzed lipid peroxidation) and cuproptosis (copper‐induced mitochondrial proteotoxicity), while exploring hypothetical “cobaltosis” as a novel pathway.
Qinghang Song, Yuxuan Yang, Lina Yang
wiley   +1 more source

Inappropriate cathepsin K secretion promotes its enzymatic activation driving heart and valve malformation

JCI Insight, 2020
Although congenital heart defects (CHDs) represent the most common birth defect, a comprehensive understanding of disease etiology remains unknown.
Po-Nien Lu, Trevor Moreland, Courtney J. Christian, Troy C. Lund, Richard A. Steet, Heather Flanagan-Steet   +5 more
doaj   +1 more source

Polyhalogenated Carbazole Impairs Dopaminergic Neurons through Dysregulation of Liquid–Liquid Phase Separation in Caenorhabditis elegans

Advanced Science, EarlyView.
Polyhalogenated carbazoles (PHCZ), persistent environmental contaminants, preferentially accumulate in neuronal tissues. Studies using Caenorhabditis elegans and human neuronal cell lines reveal that PHCZ induce dopaminergic neurodegeneration by promoting liquid–liquid phase separation of α‐synuclein, reducing condensate fluidity, impairing ...
Yuhang Luo, Hongbo Xu, Pei Peng, Xinhe Lu, Qian Zhou, Ziqing Gao, Cheng Tang, Hongyan Yin, Ying Cai, Gaga Mahai, Zhiqiang Zhu, Zuojun Liu, Hanzeng Li, Shunqing Xu   +13 more
wiley   +1 more source

Physiological pH Transition‐Driven Protein Corona Dynamics Regulate Cellular Uptake and Inflammatory Responses of Silica Nanoparticles

Advanced Science, EarlyView.
Physiological pH transitions modulate protein corona dynamics on nanoparticles, altering their cellular uptake and inflammatory responses. Acidic pHs enhance protein adsorption, induce structural changes, and promote cellular uptake. Simultaneously, inflammatory responses are reduced due to altered protein composition, including decreased ...
Yuting Ge, Fangqin Fu, Yu Gao, Tianchang He, Volker Mailänder, Daniel Crespy, Katharina Landfester, Shuai Jiang   +7 more
wiley   +1 more source

Generation and characterization of two iPSC lines derived from subjects with Free Sialic Acid Storage Disorder (FSASD)

Stem Cell Research
Free sialic acid storage disorder (FSASD) is a rare, autosomal recessive, neurodegenerative disorder caused by biallelic mutations in SLC17A5, encoding the lysosomal transmembrane sialic acid exporter, SLC17A5.
Marya S. Sabir, Petcharat Leoyklang, Mary E. Hackbarth, Evgenia Pak, Amalia Dutra, Richard Tait, Laura Pollard, David R. Adams, William A. Gahl, Marjan Huizing, May Christine V. Malicdan   +10 more
doaj   +1 more source

TWF2 Drives Tumor Progression and Sunitinib Resistance in Renal Cell Carcinoma through Hippo Signaling Suppression

Advanced Science, EarlyView.
Twinfilin actin‐binding protein (TWF2) is upregulated in sunitinib‐resistant renal cell carcinoma (RCC) cells, where it interacts with YAP and protects YAP from degradation. Stabilized YAP translocates into the nucleus and activates transcription of target genes, promoting RCC progression and drug resistance.
Liangmin Fu, Wuyuan Liao, Youyan Tan, Hansen Lin, Kun Ye, Xinwei Zhou, Mingjie Lin, Kangbo Huang, Minyu Chen, Jietao Wei, Haoqian Feng, Yuhang Chen, Jinwei Chen, Bohong Guan, Shan Li, Zhengkun Zhang, Anze Yu, Zihao Feng, Lizhen Zhang, Guannan Shu, Jun Lu, Wei Chen, Yihui Pan, Jiefeng Yang, Junhang Luo, Li Luo   +25 more
wiley   +1 more source

Nanoscale Mapping of the Subcellular Glycosylation Landscape

Advanced Science, EarlyView.
Using multiplexed super‐resolution imaging with fluorophore‐labeled lectins, this study reports intracellular glycosylation at the nanoscale across organelles and synaptic specializations. Extending glycan analysis beyond the cell surface, Glyco‐STORM reveals distinct glycosylation nanodomains in the ER, Golgi, lysosomes, and synaptic sites.
Helene Gregoria Schroeter, Steffen Sass, Mike Heilemann, Thomas Kuner, Maja Klevanski   +4 more
wiley   +1 more source

Acetylation of the Mitochondrial Chaperone GRP75 Governs ER‐Mitochondrial Calcium Homeostasis and Hepatocyte Insulin Resistance

Advanced Science, EarlyView.
Overnutrition exacerbates insulin resistance (IR) and is linked to excessive mitochondrial protein acetylation. However, it remains unclear whether and how mitochondrial protein acetylation influences IR. GCN5L1‐dependent downregulation of GRP75 acetylation at K567/612 consequently enhances ER‐mitochondrial calcium flux and induces ER stress.
Danni Wang, Jiaqi Zhang, Xinyu Yang, Qiqi Zhang, Xiuya Hu, Xin Lu, Hanni Li, Xue Bai, Kai Zhang, Michael N. Sack, Yongsheng Chang, Yingmei Wang, Lingdi Wang, Lu Zhu   +13 more
wiley   +1 more source