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The efficacy of Carbamylglutamate impacts the nutritional management of patients with N-Acetylglutamate synthase deficiency

Orphanet journal of rare diseases, 2024-04, Vol.19 (1), p.168-168

2024

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Human Naa50p (Nat5/San) Displays Both Protein Nα- and Nϵ-Acetyltransferase Activity

The Journal of biological chemistry, 2009-11, Vol.284 (45), p.31122-31129

2009

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Clinical and biochemical aspects of primary and secondary hyperammonemic disorders

Archives of biochemistry and biophysics, 2013-08, Vol.536 (2), p.101-108

Häberle, Johannes

2013

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Yeast N(alpha)-terminal acetyltransferases are associated with ribosomes

Journal of cellular biochemistry, 2008-02, Vol.103 (2), p.492-508

2008

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Inhibition of N-acetylglutamate synthase by various monocarboxylic and dicarboxylic short-chain coenzyme A esters and the production of alternative glutamate esters

Biochimica et biophysica acta, 2014-12, Vol.1842 (12), p.2510-2516

2014

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Biochemical Characterization of Hpa2 and Hpa3, Two Small Closely Related Acetyltransferases from Saccharomyces cerevisiae

The Journal of biological chemistry, 2013-07, Vol.288 (30), p.21506-21513

2013

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N-Acetylglutamate Synthase Deficiency Due to a Recurrent Sequence Variant in the N-acetylglutamate Synthase Enhancer Region

Scientific reports, 2018-10, Vol.8 (1), p.15436-7, Article 15436

2018

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Reduced expression of intestinal N-acetylglutamate synthase in suckling piglets: a novel molecular mechanism for arginine as a nutritionally essential amino acid for neonates

Amino acids, 2011-05, Vol.40 (5), p.1513-1522

2011

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Over-expression of a tomato N-acetyl-L-glutamate synthase gene (SlNAGS1) in Arabidopsis thaliana results in high ornithine levels and increased tolerance in salt and drought stresses

Journal of experimental botany, 2009-04, Vol.60 (6), p.1859-1871

2009

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Characterization of N-terminal protein modifications in Pseudomonas aeruginosa PA14

Journal of proteomics, 2015-01, Vol.114, p.214-225

2015

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Expression pattern and biochemical properties of zebrafish N-acetylglutamate synthase

PloS one, 2014-01, Vol.9 (1), p.e85597-e85597

2014

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Persistent human Borna disease virus infection modifies the acetylome of human oligodendroglia cells towards higher energy and transporter levels

Virology (New York, N.Y.), 2015-11, Vol.485, p.58-78

2015

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N-acetylglutamate synthase: structure, function and defects

Molecular genetics and metabolism, 2010, Vol.100 (Suppl 1), p.S13-S19

2010

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The N-Acetylglutamate Synthase Family: Structures, Function and Mechanisms

International journal of molecular sciences, 2015-06, Vol.16 (6), p.13004-13022

2015

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Priming of Azabicycle Biosynthesis in the Azinomycin Class of Antitumor Agents

Biochemistry (Easton), 2017-02, Vol.56 (6), p.805-808

2017

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VopA Inhibits ATP Binding by Acetylating the Catalytic Loop of MAPK Kinases

The Journal of biological chemistry, 2007-11, Vol.282 (47), p.34299-34305

2007

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A novel type of N-acetylglutamate synthase is involved in the first step of arginine biosynthesis in Corynebacterium glutamicum

BMC genomics, 2013-10, Vol.14 (1), p.713-713, Article 713

2013

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Can ornithine accumulation modulate abiotic stress tolerance in Arabidopsis?

Plant signaling & behavior, 2009-11, Vol.4 (11), p.1099-1101

2009

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A novel UPLC-MS/MS based method to determine the activity of N-acetylglutamate synthase in liver tissue

Molecular genetics and metabolism, 2016-12, Vol.119 (4), p.307-310

2016

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Down-regulation of hepatic urea synthesis by oxypurines: xanthine and uric acid inhibit N-acetylglutamate synthase

The Journal of biological chemistry, 2011-06, Vol.286 (25), p.22055-22068

2011

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The folate-coupled enzyme MTHFD2 is a nuclear protein and promotes cell proliferation

Scientific reports, 2015-10, Vol.5 (1), p.15029-15029, Article 15029

2015

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A novel biochemically salvageable animal model of hyperammonemia devoid of N-acetylglutamate synthase

Molecular genetics and metabolism, 2012-06, Vol.106 (2), p.160-168

2012

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Protein lysine acetylation in cellular function and its role in cancer manifestation

Biochimica et biophysica acta, 2010-10, Vol.1799 (10), p.702-716

2010

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Biochemical and thermodynamic analyses of Salmonella enterica Pat, a multidomain, multimeric N(ε)-lysine acetyltransferase involved in carbon and energy metabolism

mBio, 2011, Vol.2 (5)

2011

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Understanding N-Acetyl-L-Glutamate Synthase Deficiency: Mutational Spectrum, Impact of Clinical Mutations on Enzyme Functionality, and Structural Considerations

Human mutation, 2016-07, Vol.37 (7), p.679-694

2016

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The efficacy of Carbamylglutamate impacts the nutritional management of patients with N-Acetylglutamate synthase deficiency

Human Naa50p (Nat5/San) Displays Both Protein Nα- and Nϵ-Acetyltransferase Activity

Clinical and biochemical aspects of primary and secondary hyperammonemic disorders

Yeast N(alpha)-terminal acetyltransferases are associated with ribosomes

Inhibition of N-acetylglutamate synthase by various monocarboxylic and dicarboxylic short-chain coenzyme A esters and the production of alternative glutamate esters

Biochemical Characterization of Hpa2 and Hpa3, Two Small Closely Related Acetyltransferases from Saccharomyces cerevisiae

N-Acetylglutamate Synthase Deficiency Due to a Recurrent Sequence Variant in the N-acetylglutamate Synthase Enhancer Region

Reduced expression of intestinal N-acetylglutamate synthase in suckling piglets: a novel molecular mechanism for arginine as a nutritionally essential amino acid for neonates

Over-expression of a tomato N-acetyl-L-glutamate synthase gene (SlNAGS1) in Arabidopsis thaliana results in high ornithine levels and increased tolerance in salt and drought stresses

Characterization of N-terminal protein modifications in Pseudomonas aeruginosa PA14

Expression pattern and biochemical properties of zebrafish N-acetylglutamate synthase

Persistent human Borna disease virus infection modifies the acetylome of human oligodendroglia cells towards higher energy and transporter levels

N-acetylglutamate synthase: structure, function and defects

The N-Acetylglutamate Synthase Family: Structures, Function and Mechanisms

Priming of Azabicycle Biosynthesis in the Azinomycin Class of Antitumor Agents

VopA Inhibits ATP Binding by Acetylating the Catalytic Loop of MAPK Kinases

A novel type of N-acetylglutamate synthase is involved in the first step of arginine biosynthesis in Corynebacterium glutamicum

Can ornithine accumulation modulate abiotic stress tolerance in Arabidopsis?

A novel UPLC-MS/MS based method to determine the activity of N-acetylglutamate synthase in liver tissue

Down-regulation of hepatic urea synthesis by oxypurines: xanthine and uric acid inhibit N-acetylglutamate synthase

The folate-coupled enzyme MTHFD2 is a nuclear protein and promotes cell proliferation

A novel biochemically salvageable animal model of hyperammonemia devoid of N-acetylglutamate synthase

Protein lysine acetylation in cellular function and its role in cancer manifestation

Biochemical and thermodynamic analyses of Salmonella enterica Pat, a multidomain, multimeric N(ε)-lysine acetyltransferase involved in carbon and energy metabolism

Understanding N-Acetyl-L-Glutamate Synthase Deficiency: Mutational Spectrum, Impact of Clinical Mutations on Enzyme Functionality, and Structural Considerations

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