FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2076678462> ?p ?o ?g. }

• W2076678462 endingPage "115" @default.
• W2076678462 startingPage "111" @default.
• W2076678462 abstract "Hyperammonemia is less severe in arginase 1 deficiency compared with other urea cycle defects. Affected patients manifest hyperargininemia and infrequent episodes of hyperammonemia. Patients typically suffer from neurological impairment with cortical and pyramidal tract deterioration, spasticity, loss of ambulation, seizures and intellectual disability; death is less common than with other urea cycle disorders. In a mouse model of arginase I deficiency, the onset of symptoms begins with weight loss and gait instability, which progresses toward development of tail tremor with seizure-like activity; death typically occurs at about 2 weeks of life. Adeno-associated viral vector gene replacement strategies result in long-term survival of mice with this disorder. With neonatal administration of vector, the viral copy number in the liver greatly declines with hepatocyte proliferation in the first 5 weeks of life. Although the animals do survive, it is not known from a functional standpoint how well the urea cycle is functioning in the adult animals that receive adeno-associated virus. In these studies, we administered [1-13C] acetate to both littermate controls and adeno-associated virus-treated arginase 1 knockout animals and examined flux through the urea cycle. Circulating ammonia levels were mildly elevated in treated animals. Arginine and glutamine also had perturbations. Assessment 30 min after acetate administration demonstrated that ureagenesis was present in the treated knockout liver at levels as low at 3.3% of control animals. These studies demonstrate that only minimal levels of hepatic arginase activity are necessary for survival and ureagenesis in arginase-deficient mice and that this level of activity results in control of circulating ammonia. These results may have implications for potential therapy in humans with arginase deficiency." @default.
• W2076678462 created "2016-06-24" @default.
• W2076678462 creator A5000430138 @default.
• W2076678462 creator A5007155086 @default.
• W2076678462 creator A5011958310 @default.
• W2076678462 creator A5022426602 @default.
• W2076678462 creator A5027078159 @default.
• W2076678462 creator A5051034266 @default.
• W2076678462 creator A5066968727 @default.
• W2076678462 creator A5075832265 @default.
• W2076678462 date "2014-12-04" @default.
• W2076678462 modified "2023-10-16" @default.
• W2076678462 title "Minimal ureagenesis is necessary for survival in the murine model of hyperargininemia treated by AAV-based gene therapy" @default.
• W2076678462 cites W1500373302 @default.
• W2076678462 cites W1533253990 @default.
• W2076678462 cites W1969734980 @default.
• W2076678462 cites W1989184432 @default.
• W2076678462 cites W1992802718 @default.
• W2076678462 cites W1993899796 @default.
• W2076678462 cites W2000136705 @default.
• W2076678462 cites W2002601020 @default.
• W2076678462 cites W2004480433 @default.
• W2076678462 cites W2017537456 @default.
• W2076678462 cites W2019868085 @default.
• W2076678462 cites W2025771789 @default.
• W2076678462 cites W2029482313 @default.
• W2076678462 cites W2032859769 @default.
• W2076678462 cites W2038856931 @default.
• W2076678462 cites W2047816759 @default.
• W2076678462 cites W2056708356 @default.
• W2076678462 cites W2061732843 @default.
• W2076678462 cites W2062450038 @default.
• W2076678462 cites W2070177060 @default.
• W2076678462 cites W2122620803 @default.
• W2076678462 cites W2148848239 @default.
• W2076678462 cites W2171923558 @default.
• W2076678462 cites W50854289 @default.
• W2076678462 doi "https://doi.org/10.1038/gt.2014.106" @default.
• W2076678462 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/4320015" @default.
• W2076678462 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/25652179" @default.
• W2076678462 hasPublicationYear "2014" @default.
• W2076678462 type Work @default.
• W2076678462 sameAs 2076678462 @default.
• W2076678462 citedByCount "16" @default.
• W2076678462 countsByYear W20766784622015 @default.
• W2076678462 countsByYear W20766784622016 @default.
• W2076678462 countsByYear W20766784622017 @default.
• W2076678462 countsByYear W20766784622018 @default.
• W2076678462 countsByYear W20766784622019 @default.
• W2076678462 countsByYear W20766784622020 @default.
• W2076678462 countsByYear W20766784622022 @default.
• W2076678462 countsByYear W20766784622023 @default.
• W2076678462 crossrefType "journal-article" @default.
• W2076678462 hasAuthorship W2076678462A5000430138 @default.
• W2076678462 hasAuthorship W2076678462A5007155086 @default.
• W2076678462 hasAuthorship W2076678462A5011958310 @default.
• W2076678462 hasAuthorship W2076678462A5022426602 @default.
• W2076678462 hasAuthorship W2076678462A5027078159 @default.
• W2076678462 hasAuthorship W2076678462A5051034266 @default.
• W2076678462 hasAuthorship W2076678462A5066968727 @default.
• W2076678462 hasAuthorship W2076678462A5075832265 @default.
• W2076678462 hasBestOaLocation W20766784621 @default.
• W2076678462 hasConcept C104317684 @default.
• W2076678462 hasConcept C111599444 @default.
• W2076678462 hasConcept C126322002 @default.
• W2076678462 hasConcept C134018914 @default.
• W2076678462 hasConcept C164007495 @default.
• W2076678462 hasConcept C170493617 @default.
• W2076678462 hasConcept C182704531 @default.
• W2076678462 hasConcept C2776796294 @default.
• W2076678462 hasConcept C2777468819 @default.
• W2076678462 hasConcept C2778107364 @default.
• W2076678462 hasConcept C2779349466 @default.
• W2076678462 hasConcept C2781099255 @default.
• W2076678462 hasConcept C40767141 @default.
• W2076678462 hasConcept C515207424 @default.
• W2076678462 hasConcept C55493867 @default.
• W2076678462 hasConcept C71924100 @default.
• W2076678462 hasConcept C77061444 @default.
• W2076678462 hasConcept C86803240 @default.
• W2076678462 hasConcept C92087593 @default.
• W2076678462 hasConceptScore W2076678462C104317684 @default.
• W2076678462 hasConceptScore W2076678462C111599444 @default.
• W2076678462 hasConceptScore W2076678462C126322002 @default.
• W2076678462 hasConceptScore W2076678462C134018914 @default.
• W2076678462 hasConceptScore W2076678462C164007495 @default.
• W2076678462 hasConceptScore W2076678462C170493617 @default.
• W2076678462 hasConceptScore W2076678462C182704531 @default.
• W2076678462 hasConceptScore W2076678462C2776796294 @default.
• W2076678462 hasConceptScore W2076678462C2777468819 @default.
• W2076678462 hasConceptScore W2076678462C2778107364 @default.
• W2076678462 hasConceptScore W2076678462C2779349466 @default.
• W2076678462 hasConceptScore W2076678462C2781099255 @default.
• W2076678462 hasConceptScore W2076678462C40767141 @default.
• W2076678462 hasConceptScore W2076678462C515207424 @default.
• W2076678462 hasConceptScore W2076678462C55493867 @default.
• W2076678462 hasConceptScore W2076678462C71924100 @default.
• W2076678462 hasConceptScore W2076678462C77061444 @default.

• next