Results 21 to 30 of about 27,759 (158)

Genetic and functional correction of argininosuccinate lyase deficiency using CRISPR adenine base editors. [PDF]

Am J Hum Genet
Argininosuccinate lyase deficiency (ASLD) is a recessive metabolic disorder caused by variants in ASL. In an essential step in urea synthesis, ASL breaks down argininosuccinate (ASA), a pathognomonic ASLD biomarker.
Jalil S, Keskinen T, Juutila J, Sartori Maldonado R, Euro L, Suomalainen A, Lapatto R, Kuuluvainen E, Hietakangas V, Otonkoski T, Hyvönen ME, Wartiovaara K.   +11 more
europepmc   +2 more sources

Argininosuccinate lyase interacts with cyclin A2 in cytoplasm and modulates growth of liver tumor cells. [PDF]

Oncol Rep, 2017
[[abstract]]Arginine is a critical amino acid in specific cancer types including hepatocellular carcinoma (HCC) and melanoma. Novel molecular mechanisms and therapeutic targets in arginine metabolism-mediated cancer formation await further identification.
Hung YH, Huang HL, Chen WC, Yen MC, Cho CY, Weng TY, Wang CY, Chen YL, Chen LT, Lai MD.   +9 more
europepmc   +2 more sources

Biochemical testing and pathology reveal rare cause of pediatric acute liver failure: Hyperornithinemia‐hyperammonemia‐homocitrullinuria syndrome [PDF]

JPGN Reports, EarlyView.
Abstract Hyperornithinemia‐hyperammonemia‐homocitrullinuria (HHH) syndrome is a rare metabolic condition that can cause lethargy, ataxia, tachypnea, nausea, vomiting, seizures, coma, and acute liver failure. We present a 26‐month‐old female with acute liver failure who was diagnosed with HHH 1 week after admission. Histology revealed an acute hepatitic
Tierra L. Mosher, Kathryn S. Czepiel, Matthew B. Neu, Andrea Carolina Cortes Fernandez, L. Walden Browne, Annie D. Niehaus, Roberto Gugig   +6 more
wiley   +2 more sources

Understanding the role of argininosuccinate lyase transcript variants in the clinical and biochemical variability of the urea cycle disorder argininosuccinic aciduria. [PDF]

J Biol Chem, 2013
Argininosuccinic aciduria (ASA) is an autosomal recessive urea cycle disorder caused by deficiency of argininosuccinate lyase (ASL) with a wide clinical spectrum from asymptomatic to severe hyperammonemic neonatal onset life-threatening courses.
Hu L, Pandey AV, Eggimann S, Rüfenacht V, Möslinger D, Nuoffer JM, Häberle J.   +6 more
europepmc   +3 more sources

ACOD1 regulates microglial arginine metabolism and inflammatory responses [PDF]

Frontiers in Immunology
Itaconate is produced by inflammatory macrophages and promotes negative feedback on inflammation. It is synthesized by aconitate decarboxylase 1 (ACOD1) from cis-aconitate, a metabolite of the tricarboxylic acid cycle.
Eleftheria Karadima, Canelif Yilmaz, Anupam Sinha, Georgia Fodelianaki, Sofia Dimothyra, Nikolaos Nirakis, Sofia Traikov, Nicola Zamboni, Ben Wielockx, Panayotis Verginis, Mirko Peitzsch, Triantafyllos Chavakis, Vasileia Ismini Alexaki   +12 more
doaj   +2 more sources

Comparison of dietary arginine or/and inulin supplementation on growth, digestive ability and ammonia tolerance of juvenile yellow catfish Pelteobagrus fulvidraco

Aquaculture Reports, 2023
Most fish have limited use of arginine, and increasing arginine availability by adding other nutrients is an effective method. Inulin is thought to have potential synergistic effects with arginine.
Ming Li, Muzi Zhang, Haibo Jiang, Chuanjie Qin   +3 more
doaj   +1 more source

Mutation of an Arginine Biosynthesis Gene Causes Reduced Pathogenicity in Fusarium oxysporum f. sp. melonis

Molecular Plant-Microbe Interactions, 2001
Restriction enzyme-mediated integration (REMI) mutagenesis was used to tag genes required for pathogenicity of Fusarium oxysporum f. sp. melonis. Of the 1,129 REMI transformants tested, 13 showed reduced pathogenicity on susceptible melon cultivars.
Fumio Namiki, Michiko Matsunaga, Mitsuru Okuda, Iori Inoue, Kazufumi Nishi, Yoshikatsu Fujita, Takashi Tsuge   +6 more
doaj   +1 more source

Urea cycle disorders and indications for liver transplantation

Frontiers in Pediatrics, 2023
Urea cycle disorders (UCD) are inborn errors of metabolism caused by deficiency of enzymes required to convert nitrogen from ammonia into urea.
Marta García Vega, José D. Andrade, Ana Morais, Esteban Frauca, Gema Muñoz Bartolo, María D. Lledín, Ana Bergua, Loreto Hierro   +7 more
doaj   +1 more source

l-Citrulline Metabolism in Mice Augments CD4+ T Cell Proliferation and Cytokine Production In Vitro, and Accumulation in the Mycobacteria-Infected Lung

Frontiers in Immunology, 2017
Activation, recruitment, and effector function of T lymphocytes are essential for control of mycobacterial infection. These processes are tightly regulated in T cells by the availability of l-arginine within the microenvironment.
Shannon M. Lange, Shannon M. Lange, Melanie C. McKell, Melanie C. McKell, Stephanie M. Schmidt, Austin P. Hossfeld, Vandana Chaturvedi, Jeremy M. Kinder, Jaclyn W. McAlees, Ian P. Lewkowich, Sing Sing Way, Joanne Turner, Joanne Turner, Joseph E. Qualls   +13 more
doaj   +1 more source

Genetic Manipulation of Leishmania donovani to Explore the Involvement of Argininosuccinate Synthase in Oxidative Stress Management.

PLoS Neglected Tropical Diseases, 2016
Reactive oxygen and nitrogen species (ROS and RNS) produced by the phagocytic cells are the most common arsenals used to kill the intracellular pathogens.
Abul Hasan Sardar, Armando Jardim, Ayan Kumar Ghosh, Abhishek Mandal, Sushmita Das, Savita Saini, Kumar Abhishek, Ruby Singh, Sudha Verma, Ajay Kumar, Pradeep Das   +10 more
doaj   +1 more source