Results 171 to 180 of about 3,452,999 (260)

Global research dynamics in urea cycle disorders: a bibliometric study highlighting key players and future directions. [PDF]

Orphanet J Rare Dis
Wang Y, Wang X, Zhang H, Zhu B.
europepmc   +1 more source

Dual Randomness From Spatial and Temporal Variations in Stochastic Zinc Gallate Nanoclusters for PUF and TRNG Co‐Driven Image Generation

Advanced Science, EarlyView.
A dual‐randomness hardware platform is demonstrated by integrating PUF and TRNG functionalities into mixed‐ZGO nanoclusters. By leveraging spatial and temporal stochasticity, the mixed‐ZGO NCs device enables both unique identification and random signal generation.
Seungme Kang, Wangmyung Choi, Jueun Lee, Yeong Kwon Kim, Byung Chul Jang, Wonjun Shin, Hocheon Yoo   +6 more
wiley   +1 more source

Perspectives on long-term medical management of urea cycle disorders: insights from a survey of UK healthcare professionals. [PDF]

Orphanet J Rare Dis
Stepien KM, McSweeney M, Ochoa-Ferraro A, Vara R, Riley P, Smith M.   +5 more
europepmc   +1 more source

WFS1 Deficiency Impairs PIAS4‐Associated SUMOylation and Increases Ubiquitin‐Mediated Spermatogenesis‐Related Protein Degradation Leading to Testicular Male Infertility

Advanced Science, EarlyView.
The findings establish a critical role for WFS1 in human male fertility. Mechanistically, WFS1 interacts with PIAS4 to promote the SUMOylation of key spermatogenesis‐associated proteins, which in turn competitively inhibits their K48‐linked ubiquitin‐mediated degradation during spermatogenesis.
Yunchuan Tian, Guicheng Zhao, Xiang Wang, Xinyao Tang, Gan Shen, Tiechao Ruan, Chuan Jiang, Shikun Zhao, Jincheng Zhang, Xinyue Cui, Liangchai Zhuo, Jun Ma, Yihong Yang, Tingting Lin, Ying Shen   +14 more
wiley   +1 more source

Mapping challenges in the accessibility of treatment products for urea cycle disorders: A survey of European healthcare professionals. [PDF]

J Inherit Metab Dis
Stolwijk NN, Häberle J, Huidekoper HH, Wagenmakers MAEM, Hollak CEM, Bosch AM, E‐IMD and MetabERN Working Group on the Real‐World Use of products for UCD Management.   +6 more
europepmc   +1 more source

Sonogenetic Control of Engineered Probiotics for the Treatment of MASH by Modulating the Gut‐Liver Axis

Advanced Science, EarlyView.
Researchers developed an engineered probiotic encapsulated in an inulin hydrogel, enabling ultrasound‐triggered intestinal delivery of IL‐22. In MASH model mice, this system remodeled gut microbiota, strengthened the intestinal barrier and reduced portal LPS and hepatic IL‐6.
Chunya Ji, Yan Fang, Shen Zhang, Weichen Xu, Wenyun Guo, Jing Chen, Mingrui Zhu, Congjian Wen, Siming Chen, Yuli Zhu, Huixiong Xu, Haohao Yin   +11 more
wiley   +1 more source

Novel Treatment Strategy for Patients With Urea Cycle Disorders: Pharmacological Chaperones Enhance Enzyme Stability and Activity in Patient-Derived Liver Disease Models. [PDF]

J Inherit Metab Dis
Ramosaj A, Borsuk M, Underhaug J, Mathis D, Matsumoto S, Keogh A, Banz V, Pandey AV, Gougeard N, Rubio V, Martinez A, Allegri G, Poms M, Thöny B, Häberle J, Laemmle A.   +15 more
europepmc   +1 more source

Oral Actinomyces Species Exposure is Associated with Anxiety‐Like Behavior, Mitochondrial Remodeling, and Altered GABAergic Pathways

Advanced Science, EarlyView.
Oral exposure to cannabis‐associated Actinomyces species, particularly A. meyeri, is associated with anxiety‐like behavior in mice in the absence of detectable bacterial translocation or overt systemic inflammation. These findings support a potential oral‐brain metabolic axis involving candidate metabolites such as arginine and argininosuccinate ...
Tabinda Salman, Zhenwu Luo, Douglas Johnson, Arshad A. Noorani, Zhuang Wan, Bogdan Bordieanu, Zhi‐wei Ye, Rachel D. Penrod, Hongxu Xian, Sylvia Fitting, Peter W. Kalivas, Wei Jiang   +11 more
wiley   +1 more source

Understanding the Natural History and the Effects of Current Therapeutic Strategies on Urea Cycle Disorders: Insights from the UCD Spanish Registry. [PDF]

Nutrients
Martín-Hernández E, Bellusci M, Pérez-Mohand P, Correcher Medina P, Blasco-Alonso J, Morais-López A, de Las Heras J, Meavilla Olivas SM, Dougherty-de Miguel L, Couce ML, Villarroya EC, García Jiménez MC, Moreno-Lozano PJ, Vives I, Gil-Campos M, Stanescu S, Ceberio-Hualde L, Camprodón M, Cortès-Saladelafont E, López-Urdiales R, Murray Hurtado M, Márquez Armenteros AM, Sierra Córcoles C, Peña-Quintana L, Ruiz-Pons M, Alcalde C, Castellanos-Pinedo F, Dios E, Barrio-Carreras D, Martín-Cazaña M, García-Peris M, Andrade JD, García-Volpe C, de Los Santos M, García-Cazorla A, Del Toro M, Felipe-Rucián A, Comino Monroy MJ, Sánchez-Pintos P, Matas A, Gil Ortega D, Martín-Rivada Á, Bergua A, Belanger-Quintana A, Vitoria I, Yahyaoui R, Pérez B, Morales-Conejo M, Quijada-Fraile P.   +48 more
europepmc   +1 more source

Low‐Density Lipoprotein Receptor‐Targeted Biomimetic Nanoplatform Suppresses Glioblastoma Stemness and Remodels Antitumor Immunity via Dual Metabolic Disruption

Advanced Science, EarlyView.
A low‐density lipoprotein receptor‐oriented biomimetic nanoplatform couples brain‐tumor delivery with a mechanistically matched regorafenib‐erythrodiol combination. Concurrent disruption of autophagic nutrient salvage and cholesterol homeostasis collapses bioenergetic and signaling support, suppresses glioblastoma stemness, and promotes immunogenic ...
Yining Jiang, Wenzhuo Yang, Wenting Cheng, Jing Zuo, Lin Pan, Sheng Zhong, Ren Geng, Yao Zhao, Zijian Chen, Canhua Huang, Yang Bai, Liyan Zhao, Yunqian Li   +12 more
wiley   +1 more source