Carbamoyl phosphate synthase ammonia. Medical search

The monoanhydride of carbamic acid with PHOSPHORIC ACID. It is an important intermediate metabolite and is synthesized enzymatically by CARBAMYL-PHOSPHATE SYNTHASE (AMMONIA) and CARBAMOYL-PHOSPHATE SYNTHASE (GLUTAMINE-HYDROLYZING).

An enzyme that catalyzes the formation of carbamoyl phosphate from ATP, carbon dioxide, and ammonia. This enzyme is specific for arginine biosynthesis or the urea cycle. Absence or lack of this enzyme may cause CARBAMOYL-PHOSPHATE SYNTHASE I DEFICIENCY DISEASE. EC 6.3.4.16.

A colorless alkaline gas. It is formed in the body during decomposition of organic materials during a large number of metabolically important reactions. Note that the aqueous form of ammonia is referred to as AMMONIUM HYDROXIDE.

An enzyme that catalyzes the formation of carbamoyl phosphate from ATP, carbon dioxide, and glutamine. This enzyme is important in the de novo biosynthesis of pyrimidines. EC 6.3.5.5.

An enzyme that catalyzes the formation of myo-inositol-1-phosphate from glucose-6-phosphate in the presence of NAD. EC 5.5.1.4.

A urea cycle enzyme that catalyzes the formation of orthophosphate and L-citrulline (CITRULLINE) from CARBAMOYL PHOSPHATE and L-ornithine (ORNITHINE). Deficiency of this enzyme may be transmitted as an X-linked trait. EC 2.1.3.3.

An enzyme of the shikimate pathway of AROMATIC AMINO ACID biosynthesis, it generates 5-enolpyruvylshikimate 3-phosphate and ORTHOPHOSPHATE from PHOSPHOENOLPYRUVATE and shikimate-3-phosphate. The shikimate pathway is present in BACTERIA and PLANTS but not in MAMMALS.

An enzyme that catalyzes the formation of 7-phospho-2-keto-3-deoxy-D-arabinoheptonate from phosphoenolpyruvate and D-erythrose-4-phosphate. It is one of the first enzymes in the biosynthesis of TYROSINE and PHENYLALANINE. This enzyme was formerly listed as EC 4.1.2.15.

An enzyme that catalyzes the conversion of carbamoyl phosphate and L-aspartate to yield orthophosphate and N-carbamoyl-L-aspartate. (From Enzyme Nomenclature, 1992) EC 2.1.3.2.

Derivatives of carbamic acid, H2NC(=O)OH. Included under this heading are N-substituted and O-substituted carbamic acids. In general carbamate esters are referred to as urethanes, and polymers that include repeating units of carbamate are referred to as POLYURETHANES. Note however that polyurethanes are derived from the polymerization of ISOCYANATES and the singular term URETHANE refers to the ethyl ester of carbamic acid.

Inorganic salts of phosphoric acid.

An amino acid produced in the urea cycle by the splitting off of urea from arginine.

Enzymes that catalyze a reverse aldol condensation. A molecule containing a hydroxyl group and a carbonyl group is cleaved at a C-C bond to produce two smaller molecules (ALDEHYDES or KETONES). EC 4.1.2.

A class of enzymes that transfers phosphate groups and has a carboxyl group as an acceptor. EC 2.7.2.

An enzyme that catalyzes the synthesis of fructose-6-phosphate plus GLUTAMINE from GLUTAMATE plus glucosamine-6-phosphate.

Transferases are enzymes transferring a group, for example, the methyl group or a glycosyl group, from one compound (generally regarded as donor) to another compound (generally regarded as acceptor). The classification is based on the scheme "donor:acceptor group transferase". (Enzyme Nomenclature, 1992) EC 2.

Enzymes that catalyze the joining of two molecules by the formation of a carbon-nitrogen bond. EC 6.3.

An enzyme in the tryptophan biosynthetic pathway. EC 4.1.1.48.

A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.

A triazine nucleoside used as an antineoplastic antimetabolite. It interferes with pyrimidine biosynthesis thereby preventing formation of cellular nucleic acids. As the triacetate, it is also effective as an antipsoriatic.

The rate dynamics in chemical or physical systems.

A somewhat heterogeneous class of enzymes that catalyze the transfer of alkyl or related groups (excluding methyl groups). EC 2.5.

A non-essential amino acid present abundantly throughout the body and is involved in many metabolic processes. It is synthesized from GLUTAMIC ACID and AMMONIA. It is the principal carrier of NITROGEN in the body and is an important energy source for many cells.

Enzymes that catalyze the transfer of glucose from a nucleoside diphosphate glucose to an acceptor molecule which is frequently another carbohydrate. EC 2.4.1.-.

A tri-hydroxy cyclohexene carboxylic acid important in biosynthesis of so many compounds that the shikimate pathway is named after it.

Sharks of the family Squalidae, also called dogfish sharks. They comprise at least eight genera and 44 species. Their LIVER is valued for its oil and its flesh is often made into fertilizer.

A compound formed in the liver from ammonia produced by the deamination of amino acids. It is the principal end product of protein catabolism and constitutes about one half of the total urinary solids.

A simple organophosphorus compound that inhibits DNA polymerase, especially in viruses and is used as an antiviral agent.

A class of enzymes that catalyze the formation of a bond between two substrate molecules, coupled with the hydrolysis of a pyrophosphate bond in ATP or a similar energy donor. (Dorland, 28th ed) EC 6.

An enzyme that, in the course of pyrimidine biosynthesis, catalyzes ring closure by removal of water from N-carbamoylaspartate to yield dihydro-orotic acid. EC 3.5.2.3.

5'-Uridylic acid. A uracil nucleotide containing one phosphate group esterified to the sugar moiety in the 2', 3' or 5' position.

Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.

The modification of the reactivity of ENZYMES by the binding of effectors to sites (ALLOSTERIC SITES) on the enzymes other than the substrate BINDING SITES.

A rather large group of enzymes comprising not only those transferring phosphate but also diphosphate, nucleotidyl residues, and others. These have also been subdivided according to the acceptor group. (From Enzyme Nomenclature, 1992) EC 2.7.

Enzymes that catalyze the epimerization of chiral centers within carbohydrates or their derivatives. EC 5.1.3.

The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.

Derivatives of GLUTAMIC ACID. Included under this heading are a broad variety of acid forms, salts, esters, and amides that contain the 2-aminopentanedioic acid structure.

Amino-substituted glyoxylic acid derivative.

A genus of strictly anaerobic ultrathermophilic archaea, in the family THERMOCOCCACEAE, occurring in heated seawaters. They exhibit heterotrophic growth at an optimum temperature of 100 degrees C.

Enzymes that catalyze the joining of glutamine-derived ammonia and another molecule. The linkage is in the form of a carbon-nitrogen bond. EC 6.3.5.

One of the non-essential amino acids commonly occurring in the L-form. It is found in animals and plants, especially in sugar cane and sugar beets. It may be a neurotransmitter.

The parts of a macromolecule that directly participate in its specific combination with another molecule.

The key substance in the biosynthesis of histidine, tryptophan, and purine and pyrimidine nucleotides.

Models used experimentally or theoretically to study molecular shape, electronic properties, or interactions; includes analogous molecules, computer-generated graphics, and mechanical structures.

An adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.

An ester of glucose with phosphoric acid, made in the course of glucose metabolism by mammalian and other cells. It is a normal constituent of resting muscle and probably is in constant equilibrium with fructose-6-phosphate. (Stedman, 26th ed)

Purines attached to a RIBOSE and a phosphate that can polymerize to form DNA and RNA.

An essential amino acid that is physiologically active in the L-form.

Ribulose substituted by one or more phosphoric acid moieties.

The normality of a solution with respect to HYDROGEN ions; H+. It is related to acidity measurements in most cases by pH = log 1/2[1/(H+)], where (H+) is the hydrogen ion concentration in gram equivalents per liter of solution. (McGraw-Hill Dictionary of Scientific and Technical Terms, 6th ed)

Mitochondria in hepatocytes. As in all mitochondria, there are an outer membrane and an inner membrane, together creating two separate mitochondrial compartments: the internal matrix space and a much narrower intermembrane space. In the liver mitochondrion, an estimated 67% of the total mitochondrial proteins is located in the matrix. (From Alberts et al., Molecular Biology of the Cell, 2d ed, p343-4)

A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.

A group of enzymes that catalyze the transfer of carboxyl- or carbamoyl- groups. EC 2.1.3.

A compound that, along with its isomer, Cleland's reagent (DITHIOTHREITOL), is used for the protection of sulfhydryl groups against oxidation to disulfides and for the reduction of disulfides to sulfhydryl groups.

Pyrimidines with a RIBOSE and phosphate attached that can polymerize to form DNA and RNA.

Pesticides used to destroy unwanted vegetation, especially various types of weeds, grasses (POACEAE), and woody plants. Some plants develop HERBICIDE RESISTANCE.

The study of crystal structure using X-RAY DIFFRACTION techniques. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)

Azoles with an OXYGEN and a NITROGEN next to each other at the 1,2 positions, in contrast to OXAZOLES that have nitrogens at the 1,3 positions.

The facilitation of a chemical reaction by material (catalyst) that is not consumed by the reaction.

An isomer of glucose that has traditionally been considered to be a B vitamin although it has an uncertain status as a vitamin and a deficiency syndrome has not been identified in man. (From Martindale, The Extra Pharmacopoeia, 30th ed, p1379) Inositol phospholipids are important in signal transduction.

Organic salts of cyanic acid containing the -OCN radical.

Derivatives of OXALOACETIC ACID. Included under this heading are a broad variety of acid forms, salts, esters, and amides that include a 2-keto-1,4-carboxy aliphatic structure.

An enzyme of the urea cycle that catalyzes the formation of argininosuccinic acid from citrulline and aspartic acid in the presence of ATP. Absence or deficiency of this enzyme causes the metabolic disease CITRULLINEMIA in humans. EC 6.3.4.5.

A characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts.

A compound that inhibits aminobutyrate aminotransferase activity in vivo, thereby raising the level of gamma-aminobutyric acid in tissues.

The characteristic 3-dimensional shape of a protein, including the secondary, supersecondary (motifs), tertiary (domains) and quaternary structure of the peptide chain. PROTEIN STRUCTURE, QUATERNARY describes the conformation assumed by multimeric proteins (aggregates of more than one polypeptide chain).

Calcium salts of phosphoric acid. These compounds are frequently used as calcium supplements.

Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.

Antibiotic substance produced by various Streptomyces species. It is an inhibitor of enzymatic activities that involve glutamine and is used as an antineoplastic and immunosuppressive agent.

Genetically engineered MUTAGENESIS at a specific site in the DNA molecule that introduces a base substitution, or an insertion or deletion.

An enzyme that catalyzes the conversion of ATP, L-glutamate, and NH3 to ADP, orthophosphate, and L-glutamine. It also acts more slowly on 4-methylene-L-glutamate. (From Enzyme Nomenclature, 1992) EC 6.3.1.2.

Derivatives of ammonium compounds, NH4+ Y-, in which all four of the hydrogens bonded to nitrogen have been replaced with hydrocarbyl groups. These are distinguished from IMINES which are RN=CR2.

A four-carbon sugar that is found in algae, fungi, and lichens. It is twice as sweet as sucrose and can be used as a coronary vasodilator.

The insertion of recombinant DNA molecules from prokaryotic and/or eukaryotic sources into a replicating vehicle, such as a plasmid or virus vector, and the introduction of the resultant hybrid molecules into recipient cells without altering the viability of those cells.

A ureahydrolase that catalyzes the hydrolysis of arginine or canavanine to yield L-ornithine (ORNITHINE) and urea. Deficiency of this enzyme causes HYPERARGININEMIA. EC 3.5.3.1.

A species of ascomycetous fungi of the family Sordariaceae, order SORDARIALES, much used in biochemical, genetic, and physiologic studies.

Enzymes that catalyze the dehydrogenation of GLYCERALDEHYDE 3-PHOSPHATE. Several types of glyceraldehyde-3-phosphate-dehydrogenase exist including phosphorylating and non-phosphorylating varieties and ones that transfer hydrogen to NADP and ones that transfer hydrogen to NAD.

Inorganic salts that contain the -HCO3 radical. They are an important factor in determining the pH of the blood and the concentration of bicarbonate ions is regulated by the kidney. Levels in the blood are an index of the alkali reserve or buffering capacity.

Enzymes that catalyze the interconversion of aldose and ketose compounds.

Ribose substituted in the 1-, 3-, or 5-position by a phosphoric acid moiety.

Enzyme that catalyzes the first step of the tricarboxylic acid cycle (CITRIC ACID CYCLE). It catalyzes the reaction of oxaloacetate and acetyl CoA to form citrate and coenzyme A. This enzyme was formerly listed as EC 4.1.3.7.

Enzymes of the isomerase class that catalyze reactions in which a group can be regarded as eliminated from one part of a molecule, leaving a double bond, while remaining covalently attached to the molecule. (From Enzyme Nomenclature, 1992) EC 5.5.

The degree of similarity between sequences of amino acids. This information is useful for the analyzing genetic relatedness of proteins and species.

An aldotriose which is an important intermediate in glycolysis and in tryptophan biosynthesis.

Systems of enzymes which function sequentially by catalyzing consecutive reactions linked by common metabolic intermediates. They may involve simply a transfer of water molecules or hydrogen atoms and may be associated with large supramolecular structures such as MITOCHONDRIA or RIBOSOMES.

A genus of ascomycetous fungi, family Sordariaceae, order SORDARIALES, comprising bread molds. They are capable of converting tryptophan to nicotinic acid and are used extensively in genetic and enzyme research. (Dorland, 27th ed)

The five-carbon building blocks of TERPENES that derive from MEVALONIC ACID or deoxyxylulose phosphate.

Enzymes of the isomerase class that catalyze the transfer of acyl-, phospho-, amino- or other groups from one position within a molecule to another. EC 5.4.

A family of 6-membered heterocyclic compounds occurring in nature in a wide variety of forms. They include several nucleic acid constituents (CYTOSINE; THYMINE; and URACIL) and form the basic structure of the barbiturates.

A metallic element that has the atomic symbol Mg, atomic number 12, and atomic weight 24.31. It is important for the activity of many enzymes, especially those involved in OXIDATIVE PHOSPHORYLATION.

A non-essential amino acid. It is found primarily in gelatin and silk fibroin and used therapeutically as a nutrient. It is also a fast inhibitory neurotransmitter.

Derivatives of SUCCINIC ACID. Included under this heading are a broad variety of acid forms, salts, esters, and amides that contain a 1,4-carboxy terminated aliphatic structure.

A water-soluble, colorless crystal with an acid taste that is used as a chemical intermediate, in medicine, the manufacture of lacquers, and to make perfume esters. It is also used in foods as a sequestrant, buffer, and a neutralizing agent. (Hawley's Condensed Chemical Dictionary, 12th ed, p1099; McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed, p1851)

The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.

Enzymes that catalyze the cleavage of a carbon-carbon bond of a 3-hydroxy acid. (Dorland, 28th ed) EC 4.1.3.

Multicellular, eukaryotic life forms of kingdom Plantae (sensu lato), comprising the VIRIDIPLANTAE; RHODOPHYTA; and GLAUCOPHYTA; all of which acquired chloroplasts by direct endosymbiosis of CYANOBACTERIA. They are characterized by a mainly photosynthetic mode of nutrition; essentially unlimited growth at localized regions of cell divisions (MERISTEMS); cellulose within cells providing rigidity; the absence of organs of locomotion; absence of nervous and sensory systems; and an alternation of haploid and diploid generations.

Inorganic derivatives of phosphoric acid (H3PO4). Note that organic derivatives of phosphoric acids are listed under ORGANOPHOSPHATES.

The level of protein structure in which combinations of secondary protein structures (alpha helices, beta sheets, loop regions, and motifs) pack together to form folded shapes called domains. Disulfide bridges between cysteines in two different parts of the polypeptide chain along with other interactions between the chains play a role in the formation and stabilization of tertiary structure. Small proteins usually consist of only one domain but larger proteins may contain a number of domains connected by segments of polypeptide chain which lack regular secondary structure.

An enzyme that catalyzes the transfer of D-glucose from UDPglucose into 1,4-alpha-D-glucosyl chains. EC 2.4.1.11.

"Inhibition of carbamoyl-phosphate synthase (ammonia) by Tris and Hepes. Effect on Ka for N-acetylglutamate". The Biochemical ... Carbamoyl phosphate synthetase III (found in fish). Carbamoyl phosphate synthase has three main steps in its mechanism and is, ... Carbamoyl phosphate synthetase catalyzes the ATP-dependent synthesis of carbamoyl phosphate from glutamine (EC 6.3.5.5) or ... Carboxy phosphate reacts with ammonia to give carbamic acid. In turn, carbamic acid reacts with a second ATP to give carbamoyl ...

https://en.wikipedia.org/wiki/Carbamoyl_phosphate_synthetase

... such as ammonia-dependent-carbamoyl-phosphate synthase or CPS1), and inhibits its mechanism of action. The combination of ... Ammonia test, blood count, CT scan, MRI scan, electrolyte levels, genetic testing, methylmalonic acid blood test, and blood ...

https://en.wikipedia.org/wiki/Methylmalonyl-CoA_mutase_deficiency

"Carbamoyl-phosphate synthetase. Creation of an escape route for ammonia". The Journal of Biological Chemistry. 277 (42): 39722- ... Carbamoyl phosphate synthetase I Carbamoyl phosphate synthetase III Anderson PM, Meister A (December 1965). "Evidence for an ... phosphate + carbamoyl phosphate It is activated by ATP and PRPP and it is inhibited by UMP (Uridine monophosphate, the end ... phosphate + L-glutamate + carbamoyl phosphate (overall reaction) (1a) L-glutamine + H2O ⇌ {\displaystyle \rightleftharpoons } L ...

https://en.wikipedia.org/wiki/Carbamoyl_phosphate_synthase_II

... argininosuccinate synthase MeSH D08.811.464.259.350 - carbamoyl-phosphate synthase (ammonia) MeSH D08.811.464.259.400 - carbon- ... carbamoyl-phosphate synthase (glutamine-hydrolyzing) MeSH D08.811.464.259.550 - formate-tetrahydrofolate ligase MeSH D08.811. ... amide synthases MeSH D08.811.464.259.200.200 - aspartate-ammonia ligase MeSH D08.811.464.259.200.600 - glutamate-ammonia ligase ... ammonia-lyases MeSH D08.811.520.232.400.200 - aspartate ammonia-lyase MeSH D08.811.520.232.400.350 - ethanolamine ammonia-lyase ...

https://en.wikipedia.org/wiki/List_of_MeSH_codes_(D08)

In the first step ammonia is converted into carbamoyl phosphate through the investment of two ATP molecules. This step is ... The energy is used in order to rotate ATP synthase which facilitates the passage of a proton, producing ATP. A pH difference ... The urea cycle makes use of [[ornithineL-ornithine]], carbamoyl phosphate, and L-citrulline. The electron transport chain ... The urea cycle is facilitated by carbamoyl phosphate synthetase I and ornithine transcarbamylase. β-Oxidation uses pyruvate ...

https://en.wikipedia.org/wiki/Mitochondrial_matrix

As one of the urea cycle disorders, citrullinemia type I needs to be distinguished from the others: carbamoyl phosphate ... As ammonia accumulates further, the affected infant may enter a hyperammonemic coma, which indicates neurological damage and ... and N-Acetylglutamate synthase deficiency. Other diseases that may appear similar to CTLN1 include the organic acidemias and ... Signs and symptoms of CTLN1 in infants are caused by increasing levels of ammonia in the blood and cerebrospinal fluid and ...

https://en.wikipedia.org/wiki/Citrullinemia_type_I

... hyperammonemia due to carbamoyl phosphate synthetase I deficiency (carbamoyl phosphate synthetase I) Online Mendelian ... Urease producers form ammonia and carbon dioxide from urea. Ammonia then enters the systemic circulation (most venous supply of ... hyperammonemia due to N-Acetylglutamate synthase deficiency Online Mendelian Inheritance in Man (OMIM): 237300 - ... Arginase deficiency Citrullinemia N-acetylglutamate synthetase deficiency Ornithine translocase deficiency Carbamoyl phosphate ...

https://en.wikipedia.org/wiki/Hyperammonemia

N-acetyl-L-glutamate is an allosteric activator of carbamoyl phosphate synthetase, a crucial enzyme that commits NH4+ molecules ... The urea cycle gets rid of excess ammonia (NH4+) in the body, a process that must be up-regulated during times of increased ... When amino acid catabolism increases, N-Acetylglutamate synthase is up-regulated, producing more N-acetyl-L-glutamate, which up ... which down-regulates carbamoyl phosphate synthetase and the rest of the urea cycle. This response is evolutionarily ...

https://en.wikipedia.org/wiki/Aminoacylase

... carbamoyl-phosphate synthase (ammonia) EC 6.3.4.17: formate-dihydrofolate ligase EC 6.3.4.18: 5-(carboxyamino)imidazole ... carbamoyl-phosphate synthase (glutamine-hydrolysing) EC 6.3.5.6: asparaginyl-tRNA synthase (glutamine-hydrolysing) EC 6.3.5.7: ... glutathionylspermidine synthase EC 6.3.1.9: trypanothione synthase EC 6.3.1.10: adenosylcobinamide-phosphate synthase EC 6.3. ... argininosuccinate synthase EC 6.3.4.6: urea carboxylase EC 6.3.4.7: ribose-5-phosphate-ammonia ligase EC 6.3.4.8: ...

https://en.wikipedia.org/wiki/List_of_EC_numbers_(EC_6)

... or glutamine/glutamate from asymmetric dimethylarginine via DDAH Citrulline is made from ornithine and carbamoyl phosphate in ... catalyzed by nitric oxide synthase. Citrulline can be derived from: from arginine via nitric oxide synthase, as a byproduct of ... It is a key intermediate in the urea cycle, the pathway by which mammals excrete ammonia by converting it into urea. Citrulline ... "Nos2 - Nitric Oxide Synthase". Uniprot.org. Uniprot Consortium. Retrieved 10 February 2015. Cox M, Lehninger AL, Nelson DR ( ...

https://en.wikipedia.org/wiki/Citrulline

To enter the cycle, ammonia is converted to carbamoyl phosphate. The urea cycle consists of four enzymatic reactions: one ... NAcGlu is an obligate activator of carbamoyl phosphate synthetase. Synthesis of NAcGlu by N-acetylglutamate synthase (NAGS) is ... The carbamoyl phosphate then enters the urea cycle. Carbamoyl phosphate is converted to citrulline. With catalysis by ornithine ... N-Acetylglutamate synthase (NAGS) deficiency Carbamoyl phosphate synthetase (CPS) deficiency Ornithine transcarbamoylase (OTC) ...

https://en.wikipedia.org/wiki/Urea_cycle

... in which carbamoyl phosphate is produced. Carbamoyl Phosphate Synthase 1, abbreviated as CPS1, is activated by its natural ... will lead to urea cycle failure in which ammonia is not converted to urea, but rather accumulated in blood leading to the ... Carbamoyl phosphate synthase I is an enzyme found in mitochondrial matrix and it catalyzes the very first reaction of the Urea ... N-Acetylglutamate synthase deficiency is an autosomal recessive urea cycle disorder. The symptoms are visible within the first ...

https://en.wikipedia.org/wiki/N-Acetylglutamate_synthase_deficiency

CTP synthase; GMP synthase; glutamine-dependent carbamoyl-phosphate synthase; phosphoribosylformylglycinamidine synthase II; ... In molecular biology, glutamine amidotransferases (GATase) are enzymes which catalyse the removal of the ammonia group from a ... Class-I GATase domains have been found in the following enzymes: the second component of anthranilate synthase and 4-amino-4- ... Nyunoya H, Lusty CJ (August 1984). "Sequence of the small subunit of yeast carbamyl phosphate synthetase and identification of ...

https://en.wikipedia.org/wiki/Glutamine_amidotransferase

... carbamoyl-phosphate synthase (ammonia) EC 2.7.2.6: formate kinase EC 2.7.2.7: butyrate kinase EC 2.7.2.8: acetylglutamate ... sucrose-phosphate synthase EC 2.4.1.15: α,α-trehalose-phosphate synthase (UDP-forming) EC 2.4.1.16: chitin synthase EC 2.4.1.17 ... phosphate synthase EC 2.6.99.3: O-ureido-L-serine synthase 2.6.99.4: Now EC 2.3.1.234, N6-L-threonylcarbamoyladenine synthase. ... 2-hydroxy-3-oxoadipate synthase EC 2.2.1.6: acetolactate synthase EC 2.2.1.7: 1-deoxy-D-xylulose-5-phosphate synthase EC 2.2. ...

https://en.wikipedia.org/wiki/List_of_EC_numbers_(EC_2)

... enolpyruvylshikimate 3-phosphate synthase enzyme. Photosystem II Inhibitors reduce the electron flow from water to NADPH2+ ... tabaci that may cause toxic concentrations of ammonia to build up. This buildup of ammonia causes leaf chlorosis. Glycopeptides ... which catalyzes the formation of citrulline from ornithine and carbamoylphosphate in the arginine biosynthetic pathway. ...

https://en.wikipedia.org/wiki/Phytotoxin

... the enzyme carbamoyl phosphate synthase combining glutamine with CO2 in an ATP dependent reaction to form carbamoyl phosphate. ... Furthermore, the enzyme glutamine synthetase (GS) is able to transfer ammonia onto glutamate and synthesize glutamine, ... Phosphate addition to UMP is catalyzed by a kinase enzyme. The enzyme CTP synthase catalyzes the next reaction step: the ... Aspartate carbamoyltransferase condenses carbamoyl phosphate with aspartate to form uridosuccinate. Dihydroorotase performs ...

https://en.wikipedia.org/wiki/Biosynthesis

... aspartate carbamoyltransferase catalyzes a condensation reaction between aspartate and carbamoyl phosphate to form carbamoyl ... First, GTP hydrolysis fuels the addition of aspartate to IMP by adenylosuccinate synthase, substituting the carbonyl oxygen for ... and from ammonia and carbon dioxide. Recently it has been also demonstrated that cellular bicarbonate metabolism can be ... The synthesis of the pyrimidines CTP and UTP occurs in the cytoplasm and starts with the formation of carbamoyl phosphate from ...

https://en.wikipedia.org/wiki/Nucleotide

... methyl phosphate synthase EC 4.2.3.154: demethyl-4-deoxygadusol synthase EC 4.2.3.155: 2-epi-valiolone synthase EC 4.2.3.156: ... carbamoyl-serine ammonia-lyase EC 4.3.1.14: 3-aminobutyryl-CoA ammonia-lyase EC 4.3.1.15: diaminopropionate ammonia-lyase EC ... limonene synthase EC 4.1.99.11: benzylsuccinate synthase EC 4.1.99.12: 3,4-dihydroxy-2-butanone-4-phosphate synthase EC 4.1. ... deacetylipecoside synthase EC 4.3.3.5: 4′-demethylrebeccamycin synthase EC 4.3.3.6: pyridoxal 5′-phosphate synthase (glutamine ...

https://en.wikipedia.org/wiki/List_of_EC_numbers_(EC_4)

Order Human Carbamoyl-phosphate synthase ammonia mitochondrial CPS1 ELISA kit 01014537051 at Gentaur Carbamoyl-phosphate ... Order Human Carbamoyl-phosphate synthase ammonia mitochondrial CPS1 ELISA kit 01014537051 at Gentaur Carbamoyl-phosphate ... The Carbamoyl-phosphate synthase [ammonia], mitochondrial , CPS1 ELISA kit is shipped on ice packs / blue ice at +4 degrees ... This ELISA test kit for detection of Human Carbamoyl-phosphate synthase [ammonia], mitochondrial , CPS1 should be stored ...

http://amoytope.com/catalog/27497-yehua/28492-h-sapiens-carbamoyl-phosphate-synthase-ammonia-mitochondrial-carbamoyl-phosphate-synthase-1-mitochon

... carbamyl phosphate synthetase 1 (CPS1). However, despite the important role of NAGS in ammonia removal, little is known about ... N-acetylglutamate synthase (NAGS) produces a unique cofactor, N-acetylglutamate (NAG), that is essential for the catalytic ... The urea cycle converts toxic ammonia to urea within the liver of mammals. At least 6 enzymes are required for ureagenesis, ... Carbamoyl-Phosphate Synthase (Ammonia)--biosynthesis; Carbamoyl-Phosphate Synthase (Ammonia)--genetics; Gene Expression ...

https://hsrc.himmelfarb.gwu.edu/smhs_peds_facpubs/168/

Carbamoyl-Phosphate Synthase (Ammonia) Entry term(s). CP Synthase I Carbamoyl Phosphate Synthetase I Carbamoyl-Phosphate ... Carbamoyl-phosphate synthase (ammonia) Entry term(s):. CP Synthase I. Carbamoyl Phosphate Synthetase I. Carbamoyl-Phosphate ... Carbamyl Phosphate Synthase (Ammonia) Carbamyl-Phosphate Synthase (Ammonia) Synthase I, CP Synthetase I, Carbamoyl-Phosphate ... Carbamyl Phosphate Synthase (Ammonia). Carbamyl-Phosphate Synthase (Ammonia). Synthase I, CP. Synthetase I, Carbamoyl-Phosphate ...

https://decs.bvsalud.org/en/ths/resource/?id=2258&filter=ths_exact_term&q=CARBAMOYL+PHOSPHATE+SYNTHETASE+I

Description: Quantitativesandwich ELISA kit for measuring Human Carbamoyl-phosphate synthase [ammonia], mitochondrial(CPS1) in ... Description: A competitive ELISA for quantitative measurement of Human Carbamoyl phosphate synthase [ammonia], mitochondrial( ... Description: A competitive ELISA for quantitative measurement of Human Carbamoyl phosphate synthase [ammonia], mitochondrial( ... Description: A competitive ELISA for quantitative measurement of Human Carbamoyl phosphate synthase [ammonia], mitochondrial( ...

https://myelisakit.com/human-halhistidine-ammonia-lyase-elisa-kit/

Carbamoyl Phosphate Synthase (Ammonia) Deficiency Disease Carbamoyl Phosphate Synthetase I Deficiency Disease Carbamoyl- ... Carbamoyl-Phosphate Synthase I Deficiency Disease (Ornithine Carbamoyl Phosphate Deficiency) Carbamoyl-Phosphate Synthetase I ... Carbamoylphosphate Synthetase I Deficiency Disease Carbamyl Phosphate Synthetase (CPS) Deficiency Carbamyl Phosphate Synthetase ... Carbamyl Phosphate Synthetase I Deficiency Disease Carbamyl-Phosphate Synthetase I Deficiency Disease Carbohydrate Deficient ...

https://www.curehunter.com/m/keywordIndexSca.do

Carbamoyl-phosphate synthase [ammonia] mitochondrial), and transporters (hemoglobin, albumin, and UTP-glucose-1-phosphate ...

https://pesquisa.bvsalud.org/portal/?lang=pt&q=mh:Doen%C3%A7a+Hep%C3%A1tica+Induzida+por+Subst%C3%A2ncias+e+Drogas/etiologia

... carbamoyl phosphate synthetase 1 (CPS 1). The urea cycle is the bodys sole endogenous system for the clearance of ammonia.[5] ... NAG is the product of N-acetylglutamate synthase (NAGS), a mitochondrial enzyme.[6] NAGS deficiency is a very rare genetic ... fingolimod-phosphate. Fingolimod-phosphate is a non-specific sphingosine 1-phosphate (S1P) receptor modulator. With five S1P ... The dose is individually titrated to ammonia levels and symptoms. Concomitant therapy with other ammonia lowering strategies is ...

https://www.medscape.com/viewarticle/742099

Carbamoyl-phosphate synthase (ammonia) (mitochondria. GCC2bim. Glycine cleavage system lipoamide irreversible mitochondrial ...

http://bigg.ucsd.edu/models/RECON1/metabolites/nh4_m

synthase. Argininosuccinate. lyase. Carbamoyl-. phosphate. synthase. [ammonia],. mitochondrial. Glutamate. dehydrogenase. 1,. ... Carbamoyl phosphate. L-Glutamic acid. H. 2. O. NAD. Oxoglutaric acid. NADH. Oxoglutaric acid. L-Glutamic γ-semialdehyde. NAD. H ... 5-phosphate. tRNA(Arg). L-Arginyl-. tRNA(Arg). tRNA(Pro). L-Prolyl-. tRNA(Pro). Citric Acid. Cycle. Alanine. Metabolism. D- ... synthase, brain. Bifunctional. glutamate/proline-. -tRNA ligase. Fumaric acid. L-Aspartic acid. Ornithine. L-Arginine. ...

https://smpdb.ca/view/SMP0000507?highlight%5Bcompounds%5D%5B%5D=DB00148&highlight%5Bproteins%5D%5B%5D=DB00148

ammonia-lyase. Carbamoyl-. phosphate. synthase. [ammonia],. mitochondrial. Unknown. Glutamate. dehydrogenase. 1,. mitochondrial ... Carbamoyl. phosphate. Glycine. L-Glutamic acid. L-Glutamic acid. L-Aspartic acid. NH. 3. NH. 3. L-Asparagine. H. 2. O. ATP. L- ... Carbamoyl. phosphate. Glycine. L-Glutamic acid. L-Glutamic acid. L-Aspartic acid. Ammonia. Ammonia. L-Asparagine. Water. ... 5-phosphate. Biotin. Magnesium. FAD. Pyridoxal. 5-phosphate. Glycine and. Serine. Metabolism. Histidine. Metabolism. Citric ...

https://pathbank.org/view/SMP0087282

... carbamoyl phosphate synthase I and ornithine transcarbamylase. Carbamoyl phosphate synthase catalyzes the formation of 3- ... These then pass out of the cell via ATP-dependent transporters as ammonia. Ammonia is one of several organic compounds that are ... Some proteolytic enzymes can produce free ammonia directly from amino acids. Others create free ammonia indirectly by ... Ammonia, also known as NH3, is a waste product made by your body during the digestion of protein. Normally, ammonia is ...

https://www.eplifefit.com/what-is-ammonia-in-the-body/

Carbamoyl-Phosphate Synthase (Ammonia). * Immunoblotting. * Lysine. * Mass Spectrometry. * Mice. * Models, Biological. * ...

https://profiles.uchicago.edu/profiles/display/16608135

The NAGS gene provides instructions for making the enzyme N-acetylglutamate synthase. Learn about this gene and related health ... N-acetylglutamate is necessary to turn on the enzyme carbamoyl phosphate synthetase I. This enzyme controls the first step of ... As a result, excess nitrogen is not converted to urea for removal, and ammonia accumulates in the blood. The buildup of ammonia ... N-acetylglutamate synthase deficiency. More than 40 NAGS gene mutations have been identified in people with N-acetylglutamate ...

https://medlineplus.gov/genetics/gene/nags/

Argininosuccinate Synthase * Carbamoyl-Phosphate Synthase (Ammonia) [D08.811.464.259.350] Carbamoyl-Phosphate Synthase (Ammonia ... Peptide Synthases - Preferred Concept UI. M0016235. Scope note. Ligases that catalyze the joining of adjacent AMINO ACIDS by ... Peptide Synthases Entry term(s). Acid Amino Acid Ligases Acid-Amino-Acid Ligases Ligases, Acid-Amino-Acid Peptide Synthetases ...

https://decs.bvsalud.org/en/ths/resource/?id=10646&filter=ths_exact_term&q=PEPTIDE+SYNTHASES

Carbamoyl_phosphate + H+ 6.3.4.16 carbamoyl-phosphate synthase (ammonia) 6.3.5.5 carbamoyl-phosphate synthase (glutamine- ... imidazole glycerol-phosphate synthase 4.3.3.6 pyridoxal 5-phosphate synthase (glutamine hydrolysing) 6.3.4.2 CTP synthase ( ... L-aspartate + Carbamoyl_phosphate <=> H+ + L-carbamoyl-L-aspartate + phosphate 2.1.3.2 aspartate carbamoyltransferase - ... 6.3.5.5 carbamoyl-phosphate synthase (glutamine-hydrolysing) 6.3.5.6 asparaginyl-tRNA synthase (glutamine-hydrolysing) 6.3.5.7 ...

https://metano.tu-bs.de/pathway/name/UMP%20biosynthesis%20II

Ammonia is toxic to the human body and the complex process of removing it from the body is called the urea cycle. This ... These five steps can be summarized as follows: 1. Formation of Carbamoyl Phosphate: Ammonia undergoes a condensation reaction ... by the transfer of a carbamoyl group to ornithine to form citrulline this is in the presence of enzyme citrulline synthase or ... ions resulting in the formation of carbamoyl phosphate this reaction is then catalysed by the enzyme carbamoyl phosphate ...

https://www.bartleby.com/essay/Urea-Cycle-Lab-Report-FCGYFU2AZBV

1 Ammonia --toxicity. *1 Autobiography. *1 Biological Science Disciplines --.... *1 Carbamoyl-Phosphate Synthase (Amm... ... based on differential chemotactic sensitivity to cAMP and differential sensitivity to suppression of chemotaxis by ammonia.. ...

https://imsear.searo.who.int/handle/123456789/110572/simple-search?query=&sort_by=score&order=desc&rpp=10&filter_field_1=subject&filter_type_1=equals&filter_value_1=Models%2C+Biological&etal=0&filtername=dateIssued&filterquery=2007&filtertype=equals

... ligase EC 6.3.4.16 carbamoyl-phosphate synthase (ammonia) EC 6.3.4.17 formate-dihydrofolate ligase EC 6.3.4.18 5-(carboxyamino) ... EC 6.3.5.5 carbamoyl-phosphate synthase (glutamine-hydrolyse) EC 6.3.5.6 asparaginyl-tRNA synthase (glutamine-hydrolyse) EC 6.3 ... EC 6.3.1.8 glutathionylspermidine synthase EC 6.3.1.9 trypanothione synthase EC 6.3.1.10 adenosylcobinamide-phosphate synthase ... N6-hydroxylysine synthase EC 6.3.2.39 aerobactine synthase EC 6.3.2.40 cyclopeptine synthase EC 6.3.2.41 N- ...

https://fr.m.wikipedia.org/wiki/Ligase

Differential diagnoses include carbamoyl-phosphate synthetase deficiency, argininosuccinic aciduria, hyperammonemia due to N- ... Diagnosis is based on clinical manifestations and plasma ammonia levels are typically high (>200 mol/L) when encephalopathy is ... acetylglutamate synthase deficiency, citrullinemia type 1 and argininemia.. Antenatal diagnosis Prenatal diagnosis is possible ... from carbamoyl phosphate and ornithine. Mutations that abolish OTC activity completely result in the severe, neonatal-onset ...

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=664

... is a urea cycle defect that results from a deficiency in an enzyme that mediates the normal path for incorporation of ammonia. ... Molecular genetic research into carbamoyl-phosphate synthase I: molecular defects and linkage markers. J Inherit Metab Dis. ... encoded search term (Carbamoyl Phosphate Synthetase (CPS) Deficiency) and Carbamoyl Phosphate Synthetase (CPS) Deficiency What ... Carbamoyl Phosphate Synthetase (CPS) Deficiency. Updated: Jan 07, 2019 * Author: Karl S Roth, MD; Chief Editor: Maria Descartes ...

http://emedicine.medscape.com/article/942159-overview

Carbamoyl Phosphate Synthetase I use Carbamoyl-Phosphate Synthase (Ammonia). Carbamoyl-Phosphate Synthase (Ammonia) ... Carbamyl-Phosphate Synthetase I Deficiency Disease use Carbamoyl-Phosphate Synthase I Deficiency Disease ... Carbamyl Phosphate Synthase (Glutamine) use Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing). ...

https://decs.bvs.br/I/DeCS2008_Alfab-C_ing.htm

CPS1, short for Carbamoyl-Phosphate Synthase 1, provides instructions for making the enzyme carbamoyl phosphate synthetase I. ... It has been reported CPS1 is a liver-specific enzyme converting ammonia to carbamoyl phosphate in the first step of the urea ... carbamoyl-phosphate synthase 1 (CPS1), antizyme inhibitor 2 (AZIN2), dimethylglycine dehydrogenase (DMGDH), diacylglycerol ...

https://www.animbiosci.org/journal/view.php?doi=10.5713/ab.21.0569

... pentose phosphate pathway (PPP), and β-oxidation) decreased. The hepatic glycogen phosphorylation and gluconeogenesis were ... carbamoyl-phosphate synthase and argininosuccinate lyase) (Fig. 6c-d). This suggested that amino acid catabolism increased in ... 3f) and ammonia deposition (Fig. 6c) in the liver suggested that amino acids and lipids recovered from tissue apoptosis likely ... gluconate 6-phosphate and ribulose 5-phosphate) and downregulated transcription of ribulose-phosphate 3-epimerase (Fig. 5b-c), ...

https://frontiersinzoology.biomedcentral.com/articles/10.1186/s12983-020-00378-6

Carbamoyl-phosphate synthase L chain [Interproscan].","protein_coding" "CRP07177","sodB_1","Pseudomonas aeruginosa","Superoxide ... ","histidine ammonia lyase [Ensembl]. Aromatic amino acid lyase [Interproscan].","protein_coding" "AAL19829","STM0894"," ... ","phosphoenolpyruvate synthase [Ensembl]. PEP/pyruvate binding domain, mobile domain, TIM barrel domain [InterProScan]."," ... ","Glucosaminate ammonia-lyase [Ensembl]. Pyridine nucleotide-disulphide oxidoreductase [Interproscan].","protein_coding" " ...

https://bacteria.sbs.ntu.edu.sg/go/sequences/table/10511

carbamoyl phosphate synthase small subunit. Carbamoyl-phosphate synthase small chain, chloroplastic; Carbamoyl-phosphate ... Comment: This could obtain the amino group from glutamine (EC 6.3.5.5) or from ammonia (EC 6.3.4.16) ... carbamoyl phosphate synthase small subunit. carA: carbamoyl-phosphate synthase, small subunit (EC 6.3.5.5) (TIGR01368). 100%. ... 1 candidates for carA: carbamoyl phosphate synthase subunit alpha. Score. Gene. Description. Similar to. Id.. Cov.. Bits. Other ...

https://papers.genomics.lbl.gov/cgi-bin/gapView.cgi?orgs=orgsFit&set=aa&orgId=FitnessBrowser__Smeli&path=arg&step=carA

The mitochondrial carbamoyl phosphate synthetase I (CPS I) catalyses the condensation of NH4+ ions with CO2 to generate ... Toxic ammonia is transformed into harmless urea. It eliminates two waste products: ammonia and CO2. ... Argininosuccinate synthase combines with citrulline and aspartate to generate argininosuccinate. This process incorporates the ... The mitochondria include carbamoyl phosphate synthetase I and glutamate dehydrogenase. They collaborate in the creation of NH3 ...

https://www.vedantu.com/biology/urea-cycle

... is a urea cycle defect that results from a deficiency in an enzyme that mediates the normal path for incorporation of ammonia. ... Molecular genetic research into carbamoyl-phosphate synthase I: molecular defects and linkage markers. J Inherit Metab Dis. ... encoded search term (Carbamoyl Phosphate Synthetase (CPS) Deficiency) and Carbamoyl Phosphate Synthetase (CPS) Deficiency What ... Carbamoyl Phosphate Synthetase (CPS) Deficiency Medication. Updated: Jan 07, 2019 * Author: Karl S Roth, MD; Chief Editor: ...

https://emedicine.staging.medscape.com/article/942159-medication

... high carbamoyl phosphate, and low citrulline. Treatment includes ammonia-lowering medications, such as lactulose, rifaximin, ... this is an autosomal recessive disease thats caused by a deficiency in the enzyme uridine monophosphate synthase, or UMPS for ... So, deficiency of ornithine transcarbamylase results in an increase of carbamoyl phosphate in blood, which is then converted to ... Normally, ornithine transcarbamylase works in the urea cycle by combining ornithine with carbamoyl phosphate to form citrulline ...

https://www.osmosis.org/learn/Disorders_of_amino_acid_metabolism:_Pathology_review?from=/md/foundational-sciences/biochemistry-and-nutrition/biochemistry/biochemistry-and-metabolism/amino-acid-metabolism

CARBAGLU® (carglumic acid) tablets for oral suspension 200mg is a carbamoyl phosphate synthetase 1 (CPS 1) activator indicated ... Plasma ammonia levels at baseline and after treatment with CARBAGLU. *All 13 patients had abnormal ammonia levels at baseline. ... Adjunctive therapy to standard of care for the treatment of acute hyperammonemia due to N-acetylglutamate synthase (NAGS) ... Carbaglu® (carglumic acid) tablets for oral suspension 200mg is a carbamoyl phosphate synthetase 1 (CPS 1) activator indicated ...

https://www.carbaglu.com/hcp/about-carbaglu/

N-acetylglutamate synthase (NAGS) produces a unique cofactor, N-acetylglutamate (NAG), that is essential for the catalytic function of the first and rate-limiting enzyme of ureagenesis, carbamyl phosphate synthetase 1 (CPS1). (gwu.edu)
N-acetylglutamate is necessary to turn on the enzyme carbamoyl phosphate synthetase I. This enzyme controls the first step of the urea cycle, in which excess nitrogen compounds are incorporated into the cycle to be broken down. (medlineplus.gov)
This shortage of N-acetylglutamate prevents carbamoyl phosphate synthetase I from being turned on, which prevents the urea cycle from starting. (medlineplus.gov)
Formation of Carbamoyl Phosphate: Ammonia undergoes a condensation reaction with bicarbonate ions resulting in the formation of carbamoyl phosphate this reaction is then catalysed by the enzyme carbamoyl phosphate synthetase I, this step also requires Mg2+ and N-acetyl glutamate. (bartleby.com)
Differential diagnoses include carbamoyl-phosphate synthetase deficiency, argininosuccinic aciduria, hyperammonemia due to N-acetylglutamate synthase deficiency, citrullinemia type 1 and argininemia. (orpha.net)
Carbamoyl phosphate synthetase (CPS) deficiency is a urea cycle defect that results from a deficiency in an enzyme that mediates the normal path for incorporation of ammonia. (medscape.com)
at this step, N-acetylglutamate exerts its regulatory control on the mediating enzyme, carbamyl phosphate synthetase (CPS). (medscape.com)
The mitochondrial carbamoyl phosphate synthetase I (CPS I) catalyses the condensation of NH 4 + ions with CO 2 to generate carbamoyl phosphate. (vedantu.com)
Another enzyme involved in pyrimidine production, carbamoyl phosphate synthetase II (CPS-II), is found in the cytosol. (vedantu.com)
The first reaction, catalysed by Carbamoyl Phosphate Synthetase I (CPS I), is a rate-limiting or essential step in the production of urea. (vedantu.com)
The mitochondria include carbamoyl phosphate synthetase I and glutamate dehydrogenase. (vedantu.com)

An enzyme that catalyzes the formation of carbamoyl phosphate from ATP , carbon dioxide, and ammonia. (bvsalud.org)
Description: This is Double-antibody Sandwich Enzyme-linked immunosorbent assay for detection of Human Histidine Ammonia Lyase (HAL) in Tissue homogenates, cell lysates and other biological fluids. (myelisakit.com)
Description: Enzyme-linked immunosorbent assay based on the Double-antibody Sandwich method for detection of Human Histidine Ammonia Lyase (HAL) in samples from Tissue homogenates, cell lysates and other biological fluids with no significant corss-reactivity with analogues from other species. (myelisakit.com)
The NAGS gene provides instructions for making the enzyme N-acetylglutamate synthase. (medlineplus.gov)
Most NAGS gene mutations that cause N-acetylglutamate synthase deficiency change single protein building blocks (amino acids) in the N-acetylglutamate synthase enzyme. (medlineplus.gov)
Other mutations result in production of an abnormally short N-acetylglutamate synthase enzyme or prevent any enzyme from being produced at all. (medlineplus.gov)
When the function of the N-acetylglutamate synthase enzyme is reduced or eliminated, N-acetylglutamate is produced in lower-than-normal amounts, or not at all. (medlineplus.gov)
Sancho-Vaello E, Marco-Marin C, Gougeard N, Fernandez-Murga L, Rufenacht V, Mustedanagic M, Rubio V, Haberle J. Understanding N-Acetyl-L-Glutamate Synthase Deficiency: Mutational Spectrum, Impact of Clinical Mutations on Enzyme Functionality, and Structural Considerations. (medlineplus.gov)
2. Synthesis of Citrulline Carbmoyal phosphate combines with ornithine by the transfer of a carbamoyl group to ornithine to form citrulline this is in the presence of enzyme citrulline synthase or ornithine transcarbamoylase and then diffuses through the mitochondrial membrane and into the cytosol. (bartleby.com)
3. Synthesis of Argino-succinate: Once in the cytosol of the hepatocyte citrulline will comine with the ammino acid aspartate forming aginino-succinate this is catalysed by the enzyme arginine-succinate synthase. (bartleby.com)
En biochimie , une ligase est une enzyme qui catalyse la jonction de deux molécules (en anglais ligation ) par de nouvelles liaisons covalentes avec hydrolyse concomitante de l' ATP ou d'autres molécules similaires. (wikipedia.org)
Overall, activity of the cycle is regulated by the rate of synthesis of N -acetylglutamate, the enzyme activator of CPS I, which initiates incorporation of ammonia into the cycle. (medscape.com)
An inherited urea cycle disorder associated with deficiency of the enzyme ORNITHINE CARBAMOYLTRANSFERASE , transmitted as an X-linked trait and featuring elevations of amino acids and ammonia in the serum. (nih.gov)
First, dihydroxyacetone phosphate (or glycerone phosphate) from glycolysis is used by the cytosolic enzyme glycerol-3-phosphate dehydrogenase [NAD(+)] to synthesize sn-glycerol 3-phosphate. (smpdb.ca)
Second, the mitochondrial outer membrane enzyme glycerol-3-phosphate acyltransferase esterifies an acyl-group to the sn-1 position of sn-glycerol 3-phosphate to form 1-acyl-sn-glycerol 3-phosphate (lysophosphatidic acid or LPA). (smpdb.ca)
The enzyme 1-acyl-sn-glycerol-3-phosphate acyltransferase converts LPA into phosphatidic acid (1,2-diacyl-sn-glycerol 3-phosphate) by esterifying an acyl-group to the sn-2 position of the glycerol backbone. (smpdb.ca)

That's why excess levels of glutamate cause excessive amounts of ammonia production. (eplifefit.com)
For your exams, the most important recycling pathway involves pairing ammonia with alpha-ketoglutarate to form glutamate, which in turn combines with another ammonia molecule to make the amino acid glutamine. (osmosis.org)
Now in cases of hyperammonemia, or elevated blood levels of ammonia, some of the excess ammonia may combine with alpha-ketoglutarate to form glutamate, which is the main excitatory neurotransmitter in the brain. (osmosis.org)
Glutamate can then combine with another ammonia molecule to form glutamine, or with the help of vitamin B6 , it can then get converted to GABA, which is the main inhibitory neurotransmitter in the brain. (osmosis.org)
But since with hyperammonemia, there's plenty of ammonia around, more glutamate will get converted to glutamine than to GABA. (osmosis.org)

OTCD is due to mutations in the OTC gene (Xp21.1) which encodes OTC, responsible for catalyzing the synthesis of citrulline (in liver and small intestine) from carbamoyl phosphate and ornithine. (orpha.net)
Citrulline is produced by ornithine transcarbamoylase from carbamoyl phosphate and ornithine. (vedantu.com)
Argininosuccinate synthase combines with citrulline and aspartate to generate argininosuccinate. (vedantu.com)

A block distal to the formation of mitochondrial carbamoyl phosphate (CP) in the urea cycle results in excessive excretion of OA in the urine [ 1 , 2 ]. (jofem.org)

However, despite the important role of NAGS in ammonia removal, little is known about the mechanisms of its regulation. (gwu.edu)
More than 40 NAGS gene mutations have been identified in people with N-acetylglutamate synthase deficiency, which is characterized by abnormally high levels of ammonia in the blood. (medlineplus.gov)
Adjunctive therapy to standard of care for the treatment of acute hyperammonemia due to N-acetylglutamate synthase (NAGS) deficiency. (carbaglu.com)
The safety and efficacy of RAVICTI for the treatment of N-acetylglutamate synthase (NAGS) deficiency has not been established. (rxlist.com)

The buildup of ammonia damages tissues in the brain and causes the neurological problems and other signs and symptoms of N-acetylglutamate synthase deficiency. (medlineplus.gov)
Mutation analysis in patients with N-acetylglutamate synthase deficiency. (medlineplus.gov)
In patients with homozygous CPS I deficiency, the ability to fix waste nitrogen is completely absent, resulting in increasing levels of free ammonia with the attendant effects on the CNS. (medscape.com)
Chronically high levels of ornithine are associated with at least 9 inborn errors of metabolism including: Cystathionine Beta-Synthase Deficiency, Hyperornithinemia with gyrate atrophy, Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, Hyperprolinemia Type II, Lysinuric Protein Intolerance, Ornithine Aminotransferase Deficiency, Ornithine Transcarbamylase Deficiency and Prolinemia Type II. (selfdecode.com)

The urea cycle converts toxic ammonia to urea within the liver of mammals. (gwu.edu)
In the mitochondria of liver cells, the urea cycle converts excess ammonia to urea. (vedantu.com)
The urea cycle converts highly toxic ammonia to urea, which is then excreted. (vedantu.com)

Compounds comprising the urea cycle are numbered sequentially, beginning with carbamyl phosphate (1). (medscape.com)

The Carbamoyl-phosphate synthase [ammonia], mitochondrial , CPS1 ELISA kit is shipped on ice packs / blue ice at +4 degrees Celsius. (amoytope.com)
This ELISA test kit for detection of Human Carbamoyl-phosphate synthase [ammonia], mitochondrial , CPS1 should be stored refrigerated at temperatures between 2 and 8 degrees Celsius. (amoytope.com)

The nitrogens of urea come from the ammonia and aspartate, and the nitrogen in ornithine remains intact. (selfdecode.com)

Thus, causing a buildup of ammonia and toxins in the the bloodstream. (bartleby.com)

Removing the excess nitrogen prevents it from accumulating in the form of ammonia, which is toxic at high levels, especially to the brain. (medlineplus.gov)
Abstract: Ammonia is toxic to the human body and the complex process of removing it from the body is called the urea cycle. (bartleby.com)
Toxic ammonia is transformed into harmless urea. (vedantu.com)
When amino acids are metabolized , the nitrogen is formed into a toxic compound called ammonia, which is sent to the liver. (osmosis.org)
In liver cells , ammonia goes through a series of enzymatic reactions, known as the urea cycle , to be converted into the less toxic urea. (osmosis.org)

Attention cependant à ne pas confondre les enzymes synthétases avec les enzymes synthases (qui sont toutes deux des enzymes du groupe des ligases). (wikipedia.org)
Only the liver has all of the enzymes needed to generate urea from ammonia, and this route is only located in periportal hepatocytes. (vedantu.com)

They collaborate in the creation of NH 3 and its usage in the synthesis of carbamoyl phosphate. (vedantu.com)

In the liver, amino acid catabolism (transamination and urea cycle) was upregulated along with energy metabolism (TCA cycle and oxidative phosphorylation), while the carbohydrate and lipid catabolism (glycolysis, pentose phosphate pathway (PPP), and β-oxidation) decreased. (biomedcentral.com)

However, if you have an excess amount of ammonia in your system, the process will not work properly. (eplifefit.com)
As a result, excess nitrogen is not converted to urea for removal, and ammonia accumulates in the blood. (medlineplus.gov)
Ornithine plays a central role in the urea cycle and is important for the disposal of excess nitrogen (ammonia). (selfdecode.com)

Caldovic L, Morizono H, Panglao MG, Cheng SF, Packman S, Tuchman M. Null mutations in the N-acetylglutamate synthase gene associated with acute neonatal disease and hyperammonemia. (medlineplus.gov)

integrated and full-length events of MET with reactions, large generation complex PTK2( FAK1), tensin-4( TNS4) and GTPases RAP1 and RAC1, balance overwhelming peptides that undergo translation serine and result an functional surface in initial Lipid of 6-phosphate regions( Weidner et al. (evakoch.com)

Description: A sandwich ELISA kit for detection of Histidine Ammonia Lyase from Human in samples from blood, serum, plasma, cell culture fluid and other biological fluids. (myelisakit.com)
Description: A sandwich ELISA for quantitative measurement of Human L Phenylalanine ammonia lyase in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. (myelisakit.com)

terminal) nitrogen by carbamoyl phosphate. (selfdecode.com)

RECENT FINDINGS: Randomized controlled trials in adults and children demonstrate efficient lowering of parathyroid hormone (PTH) by the calcimimetics together with a reduction in serum calcium and phosphate when combined with low-dose active vitamin D, while therapy with active vitamin D analogs alone increases serum calcium and phosphate. (bvsalud.org)

DINODORNOX LIQUID TM is environmentally friendly and since it eliminates ammonia and liquefies sludge, accidental releases from collection ponds will not harm surrounding surface water. (dinatec.com)

The human body produces about 1 milliliter (ML) of ammonia per day. (eplifefit.com)

N-acetylglutamate synthase controls the production of a compound called N-acetylglutamate in the mitochondria, the energy-producing centers in cells. (medlineplus.gov)

Diagnosis is based on clinical manifestations and plasma ammonia levels are typically high (>200 mol/L) when encephalopathy is present. (orpha.net)
When every minute counts, CARBAGLU can help normalize your patients' plasma ammonia levels. (carbaglu.com)
RAVICTI is not indicated for the treatment of acute hyperammonemia in patients with UCDs because more rapidly acting interventions are essential to reduce plasma ammonia levels. (rxlist.com)
Closely monitor these patients using ammonia levels [see DOSAGE AND ADMINISTRATION ]. (rxlist.com)
It will reduce the levels of ammonia and nitrite in the water as well as the organic sludge build-up resulting from over-feeding and fish and shrimp waste. (dinatec.com)

A rare, genetic disorder of urea cycle metabolism and ammonia detoxification characterized by either a severe, neonatal-onset disease found mainly in males, or later-onset (partial) forms of the disease. (orpha.net)
The urea cycle, also known as the "Ammonia Detox Cycle", is the process by which ammonia is removed from the body. (vedantu.com)

Ammonia, also known as NH3, is a waste product made by your body during the digestion of protein. (eplifefit.com)

Now, another way for liver cells to get rid of ammonia is to recycle it back to amino acids. (osmosis.org)

When the circulation is compromised, the liver is unable to remove toxins from the bloodstream and convert the ammonia to urea. (bartleby.com)

The production of carbamoyl phosphate requires two ATPs. (vedantu.com)

Eligible hyperammonemic episodes, defined as an admission to the hospital with a plasma ammonia level ≥ 70 micromol/L, were randomized 1:1 to receive CARBAGLU or placebo, in addition to standard of care, for 7 days or until hospital discharge, whichever occurred earlier. (carbaglu.com)
The primary endpoint was time from the first dose of drug to the earlier of plasma ammonia level (carbaglu.com)
The median time to reach the primary endpoint was 1.5 days in the CARBAGLU group compared to 2.0 days in the placebo group, driven exclusively by an effect on plasma ammonia normalization. (carbaglu.com)

DINODORNOX LIQUIDT M Metabolizes the ammonia present in the waste on contact, eliminating the odors from the pens, channels and ponds. (dinatec.com)

Anatomy2

Organisms6

Chemicals and Drugs86

Analytical, Diagnostic and Therapeutic Techniques and Equipment4

Phenomena and Processes12

Information Science3

Carbamyl Phosphate

Carbamoyl-Phosphate Synthase (Ammonia)

Ammonia

Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing)

Myo-Inositol-1-Phosphate Synthase

Ornithine Carbamoyltransferase

3-Phosphoshikimate 1-Carboxyvinyltransferase

3-Deoxy-7-Phosphoheptulonate Synthase

Aspartate Carbamoyltransferase

Carbamates

Phosphates

Ornithine

Aldehyde-Lyases

Phosphotransferases (Carboxyl Group Acceptor)

Glutamine-Fructose-6-Phosphate Transaminase (Isomerizing)

Transferases

Carbon-Nitrogen Ligases

Indole-3-Glycerol-Phosphate Synthase

Citrulline

Orotic Acid

Escherichia coli

Azauridine

Kinetics

Alkyl and Aryl Transferases

Glutamine

Glucosyltransferases

Sugar Phosphates

Shikimic Acid

Dogfish

Trehalose

Urea

Phosphonoacetic Acid

Ligases

Dihydroorotase

Uridine Monophosphate

Molecular Sequence Data

Allosteric Regulation

Phosphotransferases

Carbohydrate Epimerases

Amino Acid Sequence

Glutamates

Oxamic Acid

Pyrococcus

Carbon-Nitrogen Ligases with Glutamine as Amide-N-Donor

Glutaminase

Aspartic Acid

Binding Sites

Phosphoribosyl Pyrophosphate

Models, Molecular

Adenosine Triphosphate

Glucose-6-Phosphate

Purine Nucleotides

Arginine

Ribulosephosphates

Hydrogen-Ion Concentration

Mitochondria, Liver

Pentosephosphates

Uracil Nucleotides

Phosphoenolpyruvate

Liver

Carboxyl and Carbamoyl Transferases

Dithioerythritol

Pyrimidine Nucleotides

Herbicides

Crystallography, X-Ray

Isoxazoles

Catalysis

Inositol

Cyanates

Oxaloacetates

Argininosuccinate Synthase

Substrate Specificity

Aminohydrolases

Aminooxyacetic Acid

Protein Conformation

Calcium Phosphates

Mutation

Azaserine

Mutagenesis, Site-Directed

Glutamate-Ammonia Ligase