Results 91 to 100 of about 23,457 (249)

Citrin-deficient patient-derived induced pluripotent stem cells as a pathological liver model for congenital urea cycle disorders

Molecular Genetics and Metabolism Reports
Citrin deficiency is a congenital secondary urea cycle disorder lacking useful disease models for effective treatment development. In this study, human induced pluripotent stem cells (iPSCs) were generated from two patients with citrin deficiency and ...
Mai Okano, Masahiro Yasuda, Yui Shimomura, Yoshikazu Matsuoka, Yasumasa Shirouzu, Tatsuya Fujioka, Masatoshi Kyo, Shoji Tsuji, Kazunari Kaneko, Hirofumi Hitomi   +9 more
doaj   +1 more source

Comparison of Microflow and Analytical Flow Liquid Chromatography Coupled to Mass Spectrometry Global Metabolomics Methods Using a Urea Cycle Disorder Mouse Model. [PDF]

J Proteome Res, 2022
Geller S, Lieberman H, Belanger AJ, Yew NS, Kloss A, Ivanov AR.   +5 more
europepmc   +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

New developments in the treatment of hyperammonemia: emerging use of carglumic acid

International Journal of General Medicine, 2011
Marta Daniotti1, Giancarlo la Marca2, Patrizio Fiorini1, Luca Filippi11Neonatal Intensive Care Unit, Department of Perinatal Medicine, “A. Meyer” University Children’s Hospital, Florence, Italy; 2Mass Spectrometry, Clinical ...
Marta Daniotti, Giancarlo la Marca, Patrizio Fiorini, et al   +3 more
doaj  

Recurrent Altered Mental State Associated with Nonhepatic Hyperammonemia Presented in an Elderly Female Patient: Probable Late-Onset Urea Cycle Disorder. [PDF]

J Epilepsy Res, 2021
Lee JJ, Cho S, Kim BK, Kwon O, Park JM, Lee WW, Kang K.   +6 more
europepmc   +1 more source

Inhibition of KDEL Receptors Remodels the Tumor Microenvironment for T Cell Independent Tumor Regression

Advanced Science, EarlyView.
Inhibition of KDELR2 in a small fraction of tumor cells generates sustainable immunogenic cell death conditions within the tumor microenvironment. These conditions promote the regression of tumors in a T cell independent manner. During regression, macrophages prime T cells that subsequently provide systemic protection against recurrence. The potency of
Shakti P Pattanayak, Hong Wang, Belinda Willard, Timothy A. Chan, William C Merrick, Zheng‐Rong Lu, Boaz Tirosh   +6 more
wiley   +1 more source

A Single‐Cell Transcriptomic Atlas of the Ovine Rumen Microbiome Characterizes Lineage‐Specific Metabolic Shifts Associated with Host Heat Tolerance

Advanced Science, EarlyView.
An optimized single‐cell transcriptomic framework profiles over 60 000 cells to map the ovine rumen microbiome, partitioning the ecosystem into seven cross‐species functional clusters. In heat‐resistant hosts, a lineage‐specific metabolic shift in Anaerovibrio lipolyticus toward a highly glycolytic phenotype contributes to a “nutritional sparing ...
Sanbao Zhang, Qinyang Jiang, Junjie Ma, Jianwei Zou, Fan Wang, Feifei Lv, Yanna Huang, Yongcheng Wang, Ziye Xu   +8 more
wiley   +1 more source

Targeting Lipopolysaccharide Transport Induces Membrane Lipid Remodeling and Sensitizes Acinetobacter baumannii to Colistin Treatment

Advanced Science, EarlyView.
This study identifies C4 as a lead inhibitor of the Lpt system. Notably, C4 potentiates colistin activity by disrupting LPS transport and remodeling phospholipid homeostasis, revealing a functional interplay between the Lpt and Mla systems. These findings establish a mechanistic link between Lpt inhibition and membrane lipid remodeling, positioning Lpt–
Jianya Luo, Hetianzi Zhang, Jinju Cai, Shuang Zhou, Haijie Zhang, Zhiqiang Wang, Yuan Liu   +6 more
wiley   +1 more source

Biomimetic Macrophage Cell Membrane‐Based Nanoparticles for Effective Treatment of Glioblastoma Through Boron Neutron Capture Therapy Combined With Immunotherapy

Advanced Science, EarlyView.
ABSTRACT Glioblastoma multiforme (GBM) remains largely incurable due to the blood‐brain barrier (BBB) and immunosuppressive microenvironment. While boron neutron capture therapy (BNCT) selectively eradicates tumor cells via 10B(n, α)‐7Li reactions, its clinical potential in GBM is unrealized because of the suboptimal pharmacokinetics of conventional ...
Jiawen Chen, Haoyu You, Hefa Huang, Huihui Chai, Ruize Zhu, Yun Guan, Qisheng Tang, Tianwen Li, Shan Jiang, Houshi Xu, Peng Wang, Yue Wang, Maoyuan Sun, Beining Liu, Zhen Li, Yulai Zeng, Weiqiu Ping, Yanlin Teng, Songlin Yan, Qinghui Li, Long Gu, Tao Sun, Zhifeng Shi   +22 more
wiley   +1 more source

Correcting Apparent Priming Bias Unveils Fertilizer Nitrogen‐Risk Archetypes of Surplus and Depletion Across Asian Rice Systems

Advanced Science, EarlyView.
Correcting the apparent priming effect resolves systematic biases in Asian rice fertilizer nitrogen accounting. Net soil retention drops below 7%, while 48% of fertilizer escapes, inflicting US$98.53 billion in annual reactive‐nitrogen damages. High‐resolution mapping uncovers N‐risk archetypes across 42% of the rice area, delivering a spatially ...
Xiuyun Liu, Siyuan Cai, Longlong Xia, Jagdish K. Ladha, Xiaoyuan Yan, Xu Zhao   +5 more
wiley   +1 more source