Results 161 to 170 of about 3,452,999 (260)

Impact of glycerol phenylbutyrate on biochemistry and outcomes in paediatric patients with urea cycle disorders: a multicentre case series from Saudi Arabia. [PDF]

Orphanet J Rare Dis
Hejazi R, Alghamdi TH, Salih R, Naeim Y, Althiyab H, AlAnzi T, Mohamed S, Alfadhel M, ALTassan RS, Al-Hassnan ZN, AlSayed M.   +10 more
europepmc   +1 more source

Magnetoreceptor Promotes Hepatocellular Carcinoma Development and Blunts Sorafenib‐Induced Ferroptosis by Regulating Iron Homeostasis

Advanced Science, EarlyView.
Magnetoreceptor (MagR) regulates iron homeostasis and mitochondrial function in hepatocellular carcinoma (HCC) cells. Specifically, MagR deficiency suppresses tumor cell proliferation, migration, and invasion and enhances sorafenib‐induced ferroptosis through iron accumulation.
Tao Wang, Chenghua Song, Xin He, Mengzhou Wang, Wuming Liu, Xiaolin Wang, Yuanyuan Zhang, Jia Zhang, Lin Zhang, Dan Li, Yu Zhang, Zheng Wu, Yi Lyu, Rongqian Wu   +13 more
wiley   +1 more source

Clinical characteristics and long-term outcomes of 101 patients with urea cycle disorders in China. [PDF]

Orphanet J Rare Dis
Cen Z, Ge P, Chen Y, Zhang T, Wang P, Hu L, Wu B, Huang X.   +7 more
europepmc   +1 more source

Liver Transplantation in Children with Urea Cycle Disorders: The Importance of Minimizing Waiting Time. [PDF]

Liver Transpl, 2021
Ziogas IA, Wu WK, Matsuoka LK, Pai AK, Hafberg ET, Gillis LA, Morgan TM, Alexopoulos SP.   +7 more
europepmc   +1 more source

Sarbecoviruses Suppress Innate Immunity by Recruiting PPM1A to Modulate STAT2 and ORF9b Phosphorylation

Advanced Science, EarlyView.
Sarbecovirus ORF9b recruits host phosphatase PPM1A to suppress innate immunity through dual mechanisms. PPM1A directly dephosphorylates ORF9b to sustain its activity and indirectly downregulates STAT2 phosphorylation to impair interferon signaling. Pharmacological inhibition of PPM1A restores antiviral responses and limits sarbecovirus replication ...
Lixiang Xie, Ziye Huang, Zhiyuan Zhang, Yiqiang Zhu, Xiaoqing Liu, Lei Wang, Tongyu Bi, Taizhen Liang, Jintao Lai, Meilin Hu, Guochang Qiu, Shiqi Xiao, Sen Liu, Yaoming Liu, Haiyue Rao, Tao Chen, Haojie Peng, Bin Zhang, Jia Tang, Qianying Li, Yaxin Li, Yuxin Hou, Weibo Yang, Hewei Jiang, Xiancai Ma   +24 more
wiley   +1 more source

Drug Repurposing for Treating NCOR‐Related Intellectual Disability

Advanced Science, EarlyView.
An autism‐associated NCOR1 heterozygous mutation upregulates NRGs and activates NRG backward signaling in hypothalamic GABAergic neurons in mouse models, leading to Gabra2 downregulation and cognitive deficits, which can be therapeutically targeted by repurposing FDA‐approved drugs.
Yan Kong, Wenjun Zhou, Yuxiang Sun, Zihan Huang, Zhiyue Liu, Udhaya Kumar. S, Chenyan Li, Yi Lu, Chengchuang Song, Veronica C. Ciliberto, Makenna Mack, Lana E. Kim, Zhaoyong Hu, Fang Luo, Aihua Cao, Zheng Sun   +15 more
wiley   +1 more source

P007: PP4 criteria specifications for proximal urea cycle disorders*

Genetics in Medicine Open
Kara Simpson, Nicholas Ah Mew, Ljubica Caldovic, Annette Feigenbaum, Raquel Fernandez, Emily Groopman, Andrea Gropman, Emily Kudalkar, Uta Lichter-Konecki, McKenna Kyriss, Elaine Spector, Meredith Weaver, Manya Warrier, Diane Zastrow, Amanda Thomas-Wilson   +14 more
doaj   +1 more source

A Neutrophil Membrane‐Encapsulated Pd/hCeO2 Nanoformulation for Alleviating Age‐Dependent Perioperative Neurocognitive Disorders

Advanced Science, EarlyView.
This study reports a neutrophil membrane‐encapsulated Pd/hCeO2 nanozyme (PCN) for targeted treatment of perioperative neurocognitive disorders (PND) in aged mice. PCN inhibits glycolysis‐driven microglial activation and neuroinflammation, improves mitochondrial respiration, and maintains blood‐brain barrier integrity and synaptic function, ultimately ...
Lili Huang, Jiahui Cheng, Huan Xu, Qiang Zhang, Xiao Zhang, Ye Liu, Yizhe Zhang, Wanbing Dai, Zhi Xiong, Linghuan Guo, Lingling Xu, Wen Wang, Xin Xu, Yan Zhou, Weifeng Yu, Bo Li, Diansan Su, Daxiang Wen   +17 more
wiley   +1 more source

Amino Acid Metabolism and Immune Dysfunction in Urea Cycle Disorders: T and B Cell Perspectives. [PDF]

J Inherit Metab Dis
Gemici Karaaslan B, Kiykim A, Burtecene N, Gokden M, Cansever MS, Hopurcuoglu D, Cengiz GN, Topcu B, Zubarioğlu T, Kiykim E, Cokuğras H, Aktuglu Zeybek AC.   +11 more
europepmc   +1 more source

Context‐Dependent Inverse Association of Glucose–Lipid Metabolic Status With Anemia: Evidence From Multinational Multicohort Analyses

Advanced Science, EarlyView.
Across six multinational cohorts, higher triglyceride–glucose index values are consistently associated with lower prevalent and incident anemia, despite their established adverse cardiometabolic significance. This inverse relationship varies across inflammatory, cardiovascular, and renal contexts, suggesting that glucose–lipid metabolic status carries ...
Guosong Jiang, Xinxing Wang, Lianfeng Xie, Wenna Ding, Yingyong Yin, Mei Yao, Zhibo Cui, Kaiqiang Tang, Lijie Zhong, Zhi Wang, Jia Wang, Lei Lei, Xiaoxiao Qu, Chi‐E. Qiu   +13 more
wiley   +1 more source