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n acetyl glutamate synthase

N Haskins, A Mumo, P H Brown, M Tuchman, H Morizono, L Caldovic
N-acetylglutamate synthase (NAGS; E.C. catalyzes the formation of N-acetylglutamate (NAG) from acetyl coenzyme A and glutamate. In microorganisms and plants, NAG is the first intermediate of the L-arginine biosynthesis; in animals, NAG is an allosteric activator of carbamylphosphate synthetase I and III. In some bacteria bifunctional N-acetylglutamate synthase-kinase (NAGS-K) catalyzes the first two steps of L-arginine biosynthesis. L-arginine inhibits NAGS in bacteria, fungi, and plants and activates NAGS in mammals...
December 9, 2016: Scientific Reports
I V Ryzhova, A D Nozdrachev, T V Tobias, I V Orlov, V N Chikhman, S D Solnushkin
A possible effect of nitric oxide (NO) on metabotropic glutamate receptor (mGluR) function in the amino acid afferent synapse was investigated in the isolated labyrinth of the frog Rana temporaria. The modification of the amplitude of responses of metabotropic glutamate receptor agonist trans-ACPD was analyzed during bath applied NO donor S-nitroso-N-acetyl-DL-penicillamine SNAP (0.1-100 μM) or nitric oxide synthase inhibitor L-NAME. It was shown that NO donor SNAP (1 μM) inhibited mGluR induced responses, and the inhibitor of NO-synthase L-NAME (100 μM) increased the amplitude of trans-ACPD evoked answers...
July 2016: Doklady Biological Sciences: Proceedings of the Academy of Sciences of the USSR, Biological Sciences Sections
Youngmin Kim, Mi-Jin Kwon, Jeong-Wook Choi, Min-Kyeong Lee, Chorong Kim, Jaehun Jung, Heny Aprianita, Heesop Nam, Taek-Jeong Nam
The aim of this study was to examine the anti-obesity effects of boiled tuna extract in C57BL/6N mice with obesity induced by a high-fat diet (HFD). We determined the anti-obesity effects of boiled tuna extract (100, 200, or 400 mg/kg) on the progression of HFD-induced obesity for 10 weeks. The mice were divided into 5 groups as follows: the normal diet (ND) group (n=10); the HFD group (n=10); the mice fed HFD and 100 mg/kg boiled tuna extract group (n=10); those fed a HFD and 200 mg/kg boiled tuna extract group (n=10); and those fed a HFD and 400 mg/kg boiled tuna extract group (n=10)...
October 2016: International Journal of Molecular Medicine
Xiaoqing Guo, Melanie Dumas, Bonnie L Robinson, Syed F Ali, Merle G Paule, Qiang Gu, Jyotshna Kanungo
Verapamil is a Ca(2)(+) channel blocker and is highly prescribed as an anti-anginal, antiarrhythmic and antihypertensive drug. Ketamine, an antagonist of the Ca(2)(+) -permeable N-methyl-d-aspartate-type glutamate receptors, is a pediatric anesthetic. Previously we have shown that acetyl l-carnitine (ALCAR) reverses ketamine-induced attenuation of heart rate and neurotoxicity in zebrafish embryos. Here, we used 48 h post-fertilization zebrafish embryos that were exposed to relevant drugs for 2 or 4 h. Heart beat and overall development were monitored in vivo...
February 2017: Journal of Applied Toxicology: JAT
Enea Sancho-Vaello, Clara Marco-Marín, Nadine Gougeard, Leonor Fernández-Murga, Véronique Rüfenacht, Merima Mustedanagic, Vicente Rubio, Johannes Häberle
N-acetyl-L-glutamate synthase (NAGS) deficiency (NAGSD), the rarest urea cycle defect, is clinically indistinguishable from carbamoyl phosphate synthetase 1 deficiency, rendering the identification of NAGS gene mutations key for differentiation, which is crucial, as only NAGSD has substitutive therapy. Over the last 13 years, we have identified 43 patients from 33 families with NAGS mutations, of which 14 were novel. Overall, 36 NAGS mutations have been found so far in 56 patients from 42 families, of which 76% are homozygous for the mutant allele...
July 2016: Human Mutation
Gong Cheng, Jianzhong Xu, Xiuhua Xia, Yanfeng Guo, Kai Xu, Cunsheng Su, Weiguo Zhang
A plasma jet, driven by an active helium atom supplied with an atmospheric and room temperature plasma (ARTP) biological breeding system, was used as a novel method to breed L-arginine high-yielding strains. A mutant with resistance to L-homoarginine and 8-azaguaine, ARG 3-15 (L-HA(r), 8-AG(r), L-His(-)), was screened after several rounds of screening. The L-arginine production of these mutants was more than that of the original strain, increased by 43.79% for ARG 3-15. Moreover, N-acetyl-L-glutamate synthase activity of these mutants was also increased...
July 3, 2016: Preparative Biochemistry & Biotechnology
Elden Rowland, Jitae Kim, Nazmul H Bhuiyan, Klaas J van Wijk
Protein amino (N) termini are prone to modifications and are major determinants of protein stability in bacteria, eukaryotes, and perhaps also in chloroplasts. Most chloroplast proteins undergo N-terminal maturation, but this is poorly understood due to insufficient experimental information. Consequently, N termini of mature chloroplast proteins cannot be accurately predicted. This motivated an extensive characterization of chloroplast protein N termini in Arabidopsis (Arabidopsis thaliana) using terminal amine isotopic labeling of substrates and mass spectrometry, generating nearly 14,000 tandem mass spectrometry spectra matching to protein N termini...
November 2015: Plant Physiology
Dashuang Shi, Norma M Allewell, Mendel Tuchman
N-acetylglutamate synthase (NAGS) catalyzes the production of N-acetylglutamate (NAG) from acetyl-CoA and L-glutamate. In microorganisms and plants, the enzyme functions in the arginine biosynthetic pathway, while in mammals, its major role is to produce the essential co-factor of carbamoyl phosphate synthetase 1 (CPS1) in the urea cycle. Recent work has shown that several different genes encode enzymes that can catalyze NAG formation. A bifunctional enzyme was identified in certain bacteria, which catalyzes both NAGS and N-acetylglutamate kinase (NAGK) activities, the first two steps of the arginine biosynthetic pathway...
2015: International Journal of Molecular Sciences
Gengxiang Zhao, Zhongmin Jin, Norma M Allewell, Mendel Tuchman, Dashuang Shi
Structures of the catalytic N-acetyltransferase (NAT) domain of the bifunctional N-acetyl-L-glutamate synthase/kinase (NAGS/K) from Xylella fastidiosa bound to N-acetyl-L-glutamate (NAG) with and without an N-terminal His tag have been solved and refined at 1.7 and 1.4 Å resolution, respectively. The NAT domain with an N-terminal His tag crystallized in space group P4(1)2(1)2, with unit-cell parameters a=b=51.72, c=242.31 Å. Two subunits form a molecular dimer in the asymmetric unit, which contains ∼41% solvent...
January 1, 2015: Acta Crystallographica. Section F, Structural Biology Communications
Yoshifumi Hagiya, Shotaro Kamata, Saya Mitsuoka, Norihiko Okada, Saori Yoshida, Junya Yamamoto, Rika Ohkubo, Yumi Abiko, Hidenori Yamada, Noriyuki Akahoshi, Tadashi Kasahara, Yoshito Kumagai, Isao Ishii
The key mechanism for acetaminophen hepatotoxicity is cytochrome P450 (CYP)-dependent formation of N-acetyl-p-benzoquinone imine, a potent electrophile that forms protein adducts. Previous studies revealed the fundamental role of glutathione, which binds to and detoxifies N-acetyl-p-benzoquinone imine. Glutathione is synthesized from cysteine in the liver, and N-acetylcysteine is used as a sole antidote for acetaminophen poisoning. Here, we evaluated the potential roles of transsulfuration enzymes essential for cysteine biosynthesis, cystathionine β-synthase (CBS) and cystathionine γ-lyase (CTH), in acetaminophen hepatotoxicity using hemizygous (Cbs(+/-) or Cth(+/-)) and homozygous (Cth(-/-)) knockout mice...
January 15, 2015: Toxicology and Applied Pharmacology
Liyan Hu, Carmen Diez-Fernandez, Véronique Rüfenacht, Burcu Öztürk Hismi, Özlem Ünal, Erdogan Soyucen, Mahmut Çoker, Bilge Tanyeri Bayraktar, Mehmet Gunduz, Ertugrul Kiykim, Asburce Olgac, Jordi Pérez-Tur, Vicente Rubio, Johannes Häberle
Carbamoyl phosphate synthetase 1 (CPS1) deficiency due to CPS1 mutations is a rare autosomal-recessive urea cycle disorder causing hyperammonemia that can lead to death or severe neurological impairment. CPS1 catalyzes carbamoyl phosphate formation from ammonia, bicarbonate and two molecules of ATP, and requires the allosteric activator N-acetyl-L-glutamate. Clinical mutations occur in the entire CPS1 coding region, but mainly in single families, with little recurrence. We characterized here the only currently known recurrent CPS1 mutation, p...
December 2014: Molecular Genetics and Metabolism
Kimitoshi Nakamura, Jun Kido, Hiroshi Mitsubuchi, Fumio Endo
Urea cycle disorder (UCD) is an inborn error of the metabolic pathway producing urea from ammonia, which occurs primarily in the liver. Decreased excretion of nitrogen in the urea cycle due to deficiency of carbamoyl phosphate synthase I (CPSI), ornithine transcarbamylase (OTC), argininosuccinate synthase (ASS), argininosuccinate lyase (ASL), and N-acetyl glutamate synthase (NAGS) causes hyperammonemia. We examined the clinical manifestations, treatment, and prognosis of 177 patients with UCD from January 1999 to March 2009 in Japan...
August 2014: Pediatrics International: Official Journal of the Japan Pediatric Society
Florence Gaucher-Wieczorek, Vincent Guérineau, David Touboul, Sophie Thétiot-Laurent, Franck Pelissier, Marie-Ange Badet-Denisot, Bernard Badet, Philippe Durand
Glucosamine-6-phosphate synthase (GlmS, EC catalyzes the first and rate-limiting step in the hexosamine biosynthetic pathway, leading to the synthesis of uridine-5'-diphospho-N-acetyl-D-glucosamine, the major building block for the edification of peptidoglycan in bacteria, chitin in fungi, and glycoproteins in mammals. This bisubstrate enzyme converts D-fructose-6-phosphate (Fru-6P) and L-glutamine (Gln) into D-glucosamine-6-phosphate (GlcN-6P) and L-glutamate (Glu), respectively. We previously demonstrated that matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF-MS) allows determination of the kinetic parameters of the synthase activity...
August 1, 2014: Analytical Biochemistry
Carmen Díez-Fernández, Liyan Hu, Javier Cervera, Johannes Häberle, Vicente Rubio
Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is an inborn error of the urea cycle that is due to mutations in the CPS1 gene. In the first large repertory of mutations found in CPS1D, a small CPS1 domain of unknown function (called the UFSD) was found to host missense changes with high frequency, despite the fact that this domain does not host substrate-binding or catalytic machinery. We investigate here by in vitro expression studies using baculovirus/insect cells the reasons for the prominence of the UFSD in CPS1D, as well as the disease-causing roles and pathogenic mechanisms of the mutations affecting this domain...
June 2014: Molecular Genetics and Metabolism
Ljubica Caldovic, Nantaporn Haskins, Amy Mumo, Himani Majumdar, Mary Pinter, Mendel Tuchman, Alison Krufka
The urea cycle converts ammonia, a waste product of protein catabolism, into urea. Because fish dispose ammonia directly into water, the role of the urea cycle in fish remains unknown. Six enzymes, N-acetylglutamate synthase (NAGS), carbamylphosphate synthetase III, ornithine transcarbamylase, argininosuccinate synthase, argininosuccinate lyase and arginase 1, and two membrane transporters, ornithine transporter and aralar, comprise the urea cycle. The genes for all six enzymes and both transporters are present in the zebrafish genome...
2014: PloS One
Anouk Van Leynseele, Anna Jansen, Philippe Goyens, Geert Martens, Stefaan Peeters, An Jonckheere, Linda De Meirleir
Acute hyperammonemia has a variety of etiologies and clinical manifestations. If not treated early in neonates, it leads to irreversible brain damage or death. We present a 7-day-old female patient who was brought to the emergency department with drownsiness and vomiting. Metabolic work-up revealed a blood ammonia level of 290 μmol/L (normal <100 μmol/L in neonates) with a compensated respiratory alkalosis, normal glycaemia and lactate and absence of urinary ketones. Oral feeding was stopped, an infusion of 20 % glucose was started, and sodium benzoate and arginine hydrochloride were given...
December 2014: European Journal of Pediatrics
Gang Chen, Jun Zhang, Yuzhe Zhang, Peng Liao, Tiejun Li, Lixiang Chen, Yulong Yin, Jinquan Wang, Guoyao Wu
This experiment was conducted to investigate the effects of oral administration of monosodium glutamate (MSG) on expression of genes for hepatic lipid and nitrogen metabolism in piglets. A total of 24 newborn pigs were assigned randomly into one of four treatments (n = 6/group). The doses of oral MSG administration, given at 8:00 and 18:00 to sow-reared piglets between 0 and 21 days of age, were 0 (control), 0.06 (low dose), 0.5 (intermediate dose), and 1 (high dose) g/kg body weight/day. At the end of the 3-week treatment, serum concentrations of total protein and high-density lipoprotein cholesterol in the intermediate dose group were elevated than those in the control group (P < 0...
January 2014: Amino Acids
Kathrin Petri, Frederik Walter, Marcus Persicke, Christian Rückert, Jörn Kalinowski
BACKGROUND: Arginine biosynthesis in Corynebacterium glutamicum consists of eight enzymatic steps, starting with acetylation of glutamate, catalysed by N-acetylglutamate synthase (NAGS). There are different kinds of known NAGSs, for example, "classical" ArgA, bifunctional ArgJ, ArgO, and S-NAGS. However, since C. glutamicum possesses a monofunctional ArgJ, which catalyses only the fifth step of the arginine biosynthesis pathway, glutamate must be acetylated by an as of yet unknown NAGS gene...
2013: BMC Genomics
Borimas Krutsakorn, Takashi Imagawa, Kohsuke Honda, Kenji Okano, Hisao Ohtake
BACKGROUND: Metabolic engineering has emerged as a practical alternative to conventional chemical conversion particularly in biocommodity production processes. However, this approach is often hampered by as yet unidentified inherent mechanisms of natural metabolism. One of the possible solutions for the elimination of the negative effects of natural regulatory mechanisms on artificially engineered metabolic pathway is to construct an in vitro pathway using a limited number of enzymes...
2013: Microbial Cell Factories
Gengxiang Zhao, Zhongmin Jin, Norma M Allewell, Mendel Tuchman, Dashuang Shi
N-acetylglutamate synthase (NAGS) catalyzes the conversion of AcCoA and L-glutamate to CoA and N-acetyl-L-glutamate (NAG), an obligate cofactor for carbamyl phosphate synthetase I (CPSI) in the urea cycle. NAGS deficiency results in elevated levels of plasma ammonia which is neurotoxic. We report herein the first crystal structure of human NAGS, that of the catalytic N-acetyltransferase (hNAT) domain with N-acetyl-L-glutamate bound at 2.1 Å resolution. Functional studies indicate that the hNAT domain retains catalytic activity in the absence of the amino acid kinase (AAK) domain...
2013: PloS One
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