Results 111 to 120 of about 3,452,999 (260)

α‐Synuclein Forms Distinct Micelle‐Like Assemblies at Low Ionic Strengths

Advanced Science, EarlyView.
At high ionic strength, α‐synuclein forms diverse assemblies, including oligomers, fibrils, and condensates. Here, we show that at low ionic strength, α‐synuclein adopts a distinct, low‐abundance assembly state. These assemblies maintain a constant size above a critical concentration and do not coalesce, suggesting a micelle‐like organization ...
Sophie Hertel, Soumik Ray, Federica Saraceno, Giacomo Nisini, Georgia Nasi, Thomas Oliver Mason, Antonin Kunka, Effrosyni Agrimaki, Dannie Juul Olesen, Francesco Simone Ruggeri, Alexander K. Buell   +10 more
wiley   +1 more source

Electronics‐Free Visual Monitoring of Dry Eye Disease‐Related Tear Biomarkers Using Smart Scleral Lenses

Advanced Science, EarlyView.
An electronics‐free smart scleral lens passively monitors dry eye disease‐related tear biomarkers through colorimetric sensing of chloride, pH, and ocular surface temperature. Smartphone‐assisted image analysis enables quantitative interpretation, providing a simple, non‐invasive strategy for ocular health monitoring without integrated electronics ...
Ziheng Wang, Sang Mok Park, Dawn Meyer, Seul Ah Lee, Yumin Dai, Tianhao Yu, Taewoong Park, Feiyang Li, Jason Gerard Jedlicka, Tristan Michael Long, Oluwabunmi T. Oladele, Gillian C. Shaw, Shin Ae Park, Pete S. Kollbaum, Young L. Kim, Chi Hwan Lee   +15 more
wiley   +1 more source

Plasma arginine levels in arginase deficiency in the “real world”

Molecular Genetics and Metabolism Reports
Background: Deficiency of arginase-1, the final enzyme in the urea cycle, causes a distinct clinical syndrome and is characterized biochemically by a high level of plasma arginine.
Pranoot Tanpaiboon, Yue Huang, Judy Z. Louie, Rajesh Sharma, Stephen Cederbaum, Denise Salazar   +5 more
doaj   +1 more source

Expanded newborn screening: Long term follow up of 25 patients with organic acidemias and urea cycle disorders

, 2006
Trefz FK, Töpel T, Scheible D, Frauendienst-Egger G, Hofestädt R. Expanded newborn screening: Long term follow up of 25 patients with organic acidemias and urea cycle disorders. JOURNAL OF INHERITED METABOLIC DISEASE. 2006;29(Suppl.
Trefz, F. K., Töpel, Thoralf, Scheible, D., Frauendienst-Egger, G., Hofestädt, Ralf   +4 more
core  

Oral Nanomicelle‐Mediated Sequential Butyrate Production in the Intestine for Ulcerative Colitis and Immunosuppression Treatment

Advanced Science, EarlyView.
After oral administration, inodorous GLU‐BA self‐assembles into nanomicelles in aqueous environments to resist premature gastric adsorption. Upon reaching the intestine, endogenous esterases rapidly cleave the built‐in ester bonds in these nanomicelles to release BA and GLU, with GLU further metabolized by gut microbiota to gradually generate BA ...
Feifei Xin, Zhike Xie, Danyue Zhao, Lin Yang, Yiqun Wan, Zuwei Liu, Situ Xiong, Ying Zhang, Lei Feng, Hao Wan   +9 more
wiley   +1 more source

Multi‐Technique Integration Identifies O‐GlcNAc Transferase in Fibroblast‐Like Synoviocytes as a Therapeutic Target for Rheumatoid Arthritis

Advanced Science, EarlyView.
AI‑guided single‑cell transcriptomics identifies OGT as a key regulator enriched in rheumatoid arthritis fibroblast‑like synoviocytes (RA‐FLSs). Inflammatory stimuli activate the OGT–SAP130 axis, which modulates the H3K27ac/H3K27me3 balance and epigenetically silences BTG2, promoting aggressive transformation of RA‑FLSs.
Duoli Xie, Jie Huang, Chunhao Cao, Zhenchao Tang, Xingchen Li, Jianmin Guo, Xinxin Chen, Fang Wang, Dongyi He, Jie Li, Yu Du, Bing He, Jianhua Yao, Aiping Lu, Chao Liang   +14 more
wiley   +1 more source

Arginase deficiency in Mexico: Insights from the experience of a metabolic reference center

Molecular Genetics and Metabolism Reports
Arginase deficiency (ARG1d) is an inborn error of metabolism caused by pathogenic variants in ARG1 gene, which causes a defective hydrolysis of arginine (Arg) to urea and ornithine. The molecular landscape of ARG1d in Mexico is poorly known.
M. Vela-Amieva, C. Fernández-Lainez, S. Guillén-López, L. López-Mejía, M.A. Alcántara-Ortigoza, A. González del Angel, L. Fernández-Hernández, M.E. Reyna-Fabián, B. Estandía-Ortega, I. Ibarra-González, S.W. Ryu, H. Lee   +11 more
doaj   +1 more source

D4F‐Functionalized Ceria Nanozyme‐CasRx Platform Suppresses STING and Reprograms the Renal Immune Niche in Acute Kidney Injury

Advanced Science, EarlyView.
D4F‐functionalized ceria nanozyme–CasRx nanoparticles preferentially target renal macrophages through SR‐B1‐mediated uptake in ischemia–reperfusion injury. Ceria scavenges excessive ROS, while CasRx silences STING signaling, shifting macrophages toward a less inflammatory, pro‐resolving state and increasing the proportion of regulatory T cells.
Weibo Chen, Ming Li, Xuesheng Wang, Jiajia Sun, Ziyuan Zhang, Yongfeng Gong, Benkang Shi, Nengwang Yu, Yunuo Mao, Shengtian Zhao, Xiulin Zhang, Liqiang Guo   +11 more
wiley   +1 more source

AHR‐Responsive Carbon Dots Orchestrate Skin Wound Healing and Colonic Mucosal Repair via Treg‐Mediated Macrophage Efferocytosis

Advanced Science, EarlyView.
Guided by pharmacophore modeling and molecular docking, aryl hydrocarbon receptor (AHR)‐responsive carbon dots were synthesized. They bind directly to AHR and expand gut‐resident regulatory T (Treg) cells. Treg‐derived C‐C motif chemokine ligand 1 (CCL1) enhances macrophage efferocytosis, creating a pro‐regenetative niche that promotes colonic mucosal ...
Zilu Zhu, Han Zhang, Shuai Lin, Wenqian Zheng, Xin Li, Xiaowei Xu, Ruili Yang   +6 more
wiley   +1 more source

Health-related quality of life in a systematically assessed cohort of children and adults with urea cycle disorders. [PDF]

Mol Genet Metab, 2023
Murali CN, Barber JR, McCarter R, Zhang A, Gallant N, Simpson K, Dorrani N, Wilkening GN, Hays RD, Lichter-Konecki U, Members of the Urea Cycle Disorders Consortium, Burrage LC, Nagamani SCS.   +12 more
europepmc   +1 more source