Results 111 to 120 of about 41,607 (263)

Neurometabolic disorders: urea-cycle disorder, outcomes, development and treatment

, 2008
The urea-cycle disorders (UCDs) are a group of congenital enzyme and carrier deficiencies predisposing to fatal hyperammonemia. Hyperammonemia causes alterations of neurotransmitter function and cell volume, leading to cerebral edema.
Amanda Rigas, Andrea L Gropman
core   +1 more source

Surfaceome Reprogramming of Stemsomes Promotes Lung Cancer Targeting via Potentiated Receptor–Ligand Interactions

Advanced Science, EarlyView.
This study presents a surfaceome‐reprogramming strategy for mutation‐independent lung cancer therapy by repurposing dexamethasone to prime mesenchymal stem cell‐derived nanovesicles. The engineered vesicles leverage multi‐valent interactions mediated by upregulated adhesion proteins, EPHA2, and NOTCH3.
Geunhye Kim, In Ah Kwon, Bo Seop Jeong, Hyo Seon Kim, Joo‐Hwan Park, Sung Jean Park, Sang‐Rae Lee, Youngki Lee, Dongwoo Khang   +8 more
wiley   +1 more source

Clinical, biochemical, and genotypical characteristics in urea cycle mitochondrial transporter disorders

– BACKGROUND: This study aimed to evaluate clinical, biochemical, and genotypic findings of patients diagnosed with urea cycle mitochondrial transporter disorders.
Binici, Mahir, Bilgin, Hüseyin, Tekeş, Selahattin, Bilge, Serap   +3 more
core   +1 more source

Multi‐Targeting Carnosic Acid Kills Drug‐Resistant Helicobacter pylori With Narrow‐Spectrum Activity

Advanced Science, EarlyView.
Carnosic acid selectively eradicates drug‐resistant Helicobacter pylori through a multi‐targeted mechanism involving urease inhibition, membrane disruption, and biofilm eradication. It exhibits no detectable resistance, potent in vivo efficacy, and minimal impact on gut microbiota, positioning it as a promising narrow‐spectrum lead compound against ...
Yuefan Bai, Hongming Huang, Xudong Hang, Shanwei Hu, Qian Tong, Jingchen Xu, Jia Jia, Hongkai Bi   +7 more
wiley   +1 more source

Urea cycle defects: management and outcome.

, 2005
This paper reviews the clinical presentation of 217 patients with urea cycle defects, including 121 patients with neonatal-onset forms and 96 patients with late-onset forms.
Héron, B., Nassogne, Marie-Cécile, Rabier, D., Saudubray, J M, Touati, G   +4 more
core   +1 more source

One‐Step Preparation of Lanthanide‐Doped Polyurea Multicolor Microspheres for Versatile Fluorescent Applications

Advanced Science, EarlyView.
Lanthanide‐doped polyurea microspheres are prepared via a facile one‐step precipitation polymerization. They exhibit strong hydrogen‐bonding and coordination interactions with excellent properties, including multicolor emission, high quantum yield (75.3%), remarkable stability, redispersibility, and biocompatibility.
Guiyu Zhang, Xiaoxia Yu, Xinhui Wang, Xiang Zheng Kong, Xiangling Gu, Jitao Liu, Shusheng Li, Xiaoli Zhu, Lianbao Cao, Xubao Jiang   +9 more
wiley   +1 more source

Urea cycle disorder misdiagnosed as multiple sclerosis: a case report and review of the literature

, 2017
Urea cycle disorders are a group of inborn errors of metabolism caused by dysfunction of any of the six enzymes or two transport proteins involved in urea biosynthesis.
Hussein Algahtani, Seham Alameer, Bader Shirah, Yousef Marzouk   +3 more
core   +1 more source

Electrocatalytic Coupling Conversion of Methane by Dual‐Site Control in Nickel Oxyhydroxide

Advanced Science, EarlyView.
Electrocatalytic coupling conversion of methane (CH4) is accomplished on the nickel oxyhydroxide (NiOOH), wherein the Ni─O dual‐site is triggered via the proper electronic interaction, synergistically promoting the C─H activation and C─C formation, enabling a selective and efficient C2 product generation route under ambient conditions. ABSTRACT Methane
Kailong Lu, Yangshen Chen, Junhao Wang, Yi Zhang, Sunyang He, Huimin Xu, Hui Liu, Jiao Deng   +7 more
wiley   +1 more source

Are protein substitutes available in Italy for infants with inherited metabolic diseases all the same?

Frontiers in Nutrition
IntroductionInherited metabolic diseases (IMDs) represent a major clinical challenge, especially during the neonatal and infant periods. They require tailored and long-term nutritional management to ensure proper growth and development.
Margherita Di Costanzo, Margherita Di Costanzo, Martina Tosi, Martina Tosi, Martina Muzi, Enrico Vito Buono, Valentina D’Onghia, Anna Munerati, Gabriele Tarditi, Simone Bruni, Elvira Verduci, Elvira Verduci, Giacomo Biasucci, Giacomo Biasucci   +13 more
doaj   +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