Results 111 to 120 of about 129,105 (287)

Chaperone-Like Therapy with Tetrahydrobiopterin in Clinical Trials for Phenylketonuria: Is Genotype a Predictor of Response? [PDF]

, 2011
Prospectively enrolled phenylketonuria patients (n=485) participated in an international Phase II clinical trial to identify the prevalence of a therapeutic response to daily doses of sapropterin dihydrochloride (sapropterin, KUVAN(®)). Responsive patients were then enrolled in two subsequent Phase III clinical trials to examine safety, ability to ...
Christineh N, Sarkissian, Alejandra, Gamez, Patrick, Scott, Jerome, Dauvillier, Alejandro, Dorenbaum, Charles R, Scriver, Raymond C, Stevens   +6 more
openaire   +2 more sources

Targeting Lactate and Lactylation in Cancer Metabolism and Immunotherapy

Advanced Science, EarlyView.
Lactate, once deemed a metabolic waste, emerges as a central regulator of cancer progression. This review elucidates how lactate and its epigenetic derivative, protein lactylation, orchestrate tumor metabolism, immune suppression, and therapeutic resistance.
Jiajing Gong, Yuhang Xie, Zhijie Weng, Yongzhi Wu, Longjiang Li, Bo Li   +5 more
wiley   +1 more source

Alkaline Phosphatase‐Activated NIR‐II AIEgens Nanosystem for Surgical and Postoperative Closed‐Loop Therapy of Advanced Osteosarcoma

Advanced Science, EarlyView.
This study designed a tumor microenvironment‐responsive AIEgen nanoparticle for near‐infrared photoimmunotherapy, which inhibits primary tumors and metastasis by promoting pyroptosis and suppressing aerobic glycolysis. ABSTRACT In advanced osteosarcoma, tumor invasion often prevents complete resection, and immunotherapy is limited by the tumor's ...
Kaiyuan Liu, Ruotong Li, Li Zhang, Hengli Lu, Binhui Yang, Qian Hu, Yining Tao, Haoran Mu, Jing Han, Pengfei Zan, Jiakang Shen, Dongqing Zuo, Hongsheng Wang, Wei Sun, Xingjun Zhu   +14 more
wiley   +1 more source

Hepatic cytochromes P450: structural degrons and barcodes, posttranslational modifications and cellular adapters in the ERAD-endgame. [PDF]

, 2016
The endoplasmic reticulum (ER)-anchored hepatic cytochromes P450 (P450s) are enzymes that metabolize endo- and xenobiotics i.e. drugs, carcinogens, toxins, natural and chemical products.
Correia, Maria Almira, Kim, Sung-Mi, Liu, Yi, Nabavi, Noushin, Wang, YongQiang   +4 more
core   +1 more source

3D Bioprinted Fat‐Myocardium Model Unravels the Role of Adipocyte Hypertrophy in Atrial Dysfunction

Advanced Science, EarlyView.
A human‐derived 3D bioprinted fat–myocardium model is developed to investigate how adipocyte hypertrophy drives atrial dysfunction in obesity. Palmitate‐induced adipocyte hypertrophy promotes adipose dysfunction that impairs atrial cardiomyocyte metabolism and electrophysiology through both paracrine and direct interactions. This platform recapitulates
Lara Ece Celebi, Pinar Zorlutuna
wiley   +1 more source

Altered chaperone–nonmuscle myosin II interactions drive pathogenicity of the UNC45A c.710T>C variant in osteo-oto-hepato-enteric syndrome

JCI Insight
The osteo-oto-hepato-enteric (O2HE) syndrome is a severe autosomal recessive disease ascribed to loss-of-function mutations in the Unc-45 myosin chaperone A (UNC45A) gene. The clinical spectrum includes bone fragility, hearing loss, cholestasis, and life-
Stephanie Waich, Karin Kreidl, Julia Vodopiutz, Arzu Meltem Demir, Adam R. Pollio, Vojtěch Dostál, Kristian Pfaller, Marianna Parlato, Nadine Cerf-Bensussan, Rüdiger Adam, Georg F. Vogel, Holm H. Uhlig, Frank M. Ruemmele, Thomas Müller, Michael W. Hess, Andreas R. Janecke, Lukas A. Huber, Taras Valovka   +17 more
doaj   +1 more source

Role of Glucose-regulated Protein 78 in Embryonic Development and Neurological Disorders

Journal of the Formosan Medical Association, 2011
Glucose-regulated protein 78 (GRP78) is an important chaperone protein that is predominantly expressed in the endoplasmic reticulum. The multifunctional roles of GRP78 in protein folding, endoplasmic retic-ulum calcium binding, cytoprotection, and anti ...
Wen-Chin Weng, Wang-Tso Lee, Wen-Ming Hsu, Bei-En Chang, Hsinyu Lee   +4 more
doaj   +1 more source

Alphaviral Capsid Proteins Inhibit Stress Granule Assembly via Competitive RNA Binding With G3BP1

Advanced Science, EarlyView.
Stress granules exert antiviral functions. This study illustrates a conserved function of alphaviral capsid proteins in modulating stress granules. Oligomerization mediated by a helical motif coupled with a positively charged intrinsically disordered region (IDR) directly competes with G3BP1 for RNA binding, thereby disrupting G3BP1‐RNA liquid–liquid ...
Yun Zhang, Yi Liu, Zhiying Yao, Haolong Lai, Xiaoxin Chen, Ziqiu Wang, Yiqiong Bao, Tingting Li, Xiaoming Zhou, Xiabin Chen, Peiguo Yang   +10 more
wiley   +1 more source

Adaptive response of neonatal sepsis-derived Group B Streptococcus to bilirubin [PDF]

, 2018
This work was funded by the Neonatal Unit Endowment Fund, Aberdeen Maternity Hospital. RH is funded by a career researcher fellowship from NHS Research Scotland. SG was funded by the MRC Flagship PhD programme.
Berry, Susan, Collie-Duguid, Elaina S. R., De Paiva Alves, Eduardo, El-Omar, Emad, Gibson, Sophie, Goddard, Mark, Hansen, Richard, Hold, Georgina L., Karcher, Anne Marie, MacLaren, Andrew, Munro, Mike   +10 more
core   +4 more sources

Mitochondrial CircRNA CircMT‐RNR2 Safeguards Antioxidant Defense to Support Fibroblast Functions in Wound Repair

Advanced Science, EarlyView.
CircMT‐RNR2 promotes wound healing by enhancing fibroblast proliferation, migration, contraction, and extracellular matrix (ECM) production. It sustains mitochondrial redox balance by stabilizing the antioxidant protein PRDX3, thereby reducing ROS‐induced damage. In diabetic foot ulcers, hypoxia, hyperglycemia, and reduced FGF2 and EGF expression lower
Guanglin Niu, Jennifer Geara, Yongjian Chen, Lihua Luo, Yanwei Xiao, Zhuang Liu, Margaux Gaborieau, Ling Pan, Edmund Loh, Dongqing Li, Pehr Sommar, Aoxue Wang, Xiaowei Zheng, Ning Xu Landén   +13 more
wiley   +1 more source