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" (PDF). Biochem. J. ... Carbamoyl phosphate synthetase catalyzes the ATP-dependent synthesis of carbamoyl phosphate from glutamine (EC 6.3.5.5) or ... Carbamoyl phosphate synthetase I (mitochondria, urea cycle). *Carbamoyl phosphate synthetase II (cytosol, pyrimidine metabolism ... Carboxy phosphate reacts with ammonia to give carbamic acid. In turn, carbamic acid reacts with a second ATP to give carbamoyl ...
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 ...
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) ...
... is made from ornithine and carbamoyl phosphate in one of the central reactions in the urea cycle. It is also ... "Nos2 - Nitric Oxide Synthase". Uniprot.org. Uniprot Consortium. Retrieved 10 February 2015.. ... the pathway by which mammals excrete ammonia by converting it into urea. Citrulline is also produced as a byproduct of the ... enzymatic production of nitric oxide from the amino acid arginine, catalyzed by nitric oxide synthase.[4] ...
Carbamoyl phosphate synthetase I. *Ornithine transcarbamylase. *N-Acetylglutamate synthase. alcohol metabolism. *ALDH2 ... pyridoxal phosphate binding. • hydrolase activity. • 3-hydroxykynureninase activity. Cellular component. • cytosol. • ... phosphate (PLP) dependent enzymes. To date, two structures of human kynureninase have determined by X-ray diffraction with ...
Carbamoyl phosphate synthetase I. *Ornithine transcarbamylase. *N-Acetylglutamate synthase. alcohol metabolism. *ALDH2 ...
Carbamoyl phosphate synthetase I deficiency. *Citrullinemia. *N-Acetylglutamate synthase deficiency. *Ornithine ... Biomarin is currently conducting clinical trials to investigate PEG-PAL (PEGylated recombinant phenylalanine ammonia lyase or ' ...
Carbamoyl phosphate synthetase I deficiency. *Citrullinemia. *N-Acetylglutamate synthase deficiency. *Ornithine ... The gene that encodes for Histidine ammonia-lyase (HAL) spans a roughly 25 kb and consists of 21 exons[4] located at the 12q22- ... as the result of an inborn error of metabolism that may result in either an inactive or a severely reduced Histidine ammonia- ...
What is carbamoyl-phosphate synthase 1, mitochondrial? Meaning of carbamoyl-phosphate synthase 1, mitochondrial medical term. ... Looking for online definition of carbamoyl-phosphate synthase 1, mitochondrial in the Medical Dictionary? carbamoyl-phosphate ... carbamoyl phosphate synthetases. *carbamoyl-phosphate synthase [ammonia], mitochondrial. *carbamoyl-phosphate synthase 1, ... Carbamoyl-phosphate synthase 1, mitochondrial , definition of carbamoyl-phosphate synthase 1, mitochondrial by Medical ...
Co?expression of the carbamoyl?phosphate synthase 1 gene and its long non?coding RNA correlates with poor prognosis of patients ... The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. This ... 4217C,A polymorphism in carbamoyl-phosphate synthase 1 gene may not associate with hyperammonemia development during valproic ... The Study of Carbamoyl Phosphate Synthetase 1 Deficiency Sheds Light on the Mechanism for Switching On/Off the Urea Cycle.. ...
Compare carbamoyl-phosphate synthase 1 ELISA Kits from leading suppliers on Biocompare. View specifications, prices, citations ... Human Carbamoyl-phosphate synthase [ammonia], mitochondrial, CPS1 ELISA Kit *. Detection Target: carbamoyl-phosphate synthetase ... carbamoyl-phosphate synthase 1 ELISA Kits. The ELISA (enzyme-linked immunosorbent assay) is a well-established antibody-based ... Your search returned 15 carbamoyl-phosphate synthase 1 ELISA ELISA Kit across 4 suppliers. ...
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* * Cyclic AMP ... carbamyl phosphate synthetase 1 (CPS1). However, despite the important role of NAGS in ammonia removal, little is known about ... Transcriptional regulation of N-acetylglutamate synthase PLoS One. 2012;7(2):e29527. doi: 10.1371/journal.pone.0029527. Epub ...
The amino acid sequences of all available proteins that catalyze the formation of carbamoylphosphate were retrieved from ... Carbamoylphosphate is a common intermediate in the metabolic pathways leading to the biosynthesis of arginine and pyrimidines. ... Carbamoyl-Phosphate Synthase (Ammonia) / genetics* * Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing) / genetics* * ... In gram-negative bacteria carbamoylphosphate is synthesized by a two-subunit enzyme with glutaminase and carbamoylphosphate ...
carbamoyl-phosphate synthase (ammonia) (1) * carbohydrate restricted diet (1) * carbohydrates (1) * cd95 antigens (1) ... TOPICS: lung transplantation, glutamate-ammonia ligase, hyperammonemia, glutamine synthetase deficiency Ann Intern Med. 1997; ...
Carbamoyl phosphate synthase [ammonia] mitochondrial antibody. *Carbamoyl phosphate synthase antibody. *Carbamoyl phosphate ... Co-expression of the carbamoyl-phosphate synthase 1 gene and its long non-coding RNA correlates with poor prognosis of patients ... Carbamoyl phosphate synthetase I antibody. *Carbamoyl-phosphate synthase [ammonia] antibody. *Carbamoyl-phosphate synthetase I ... Defects in CPS1 are the cause of carbamoyl phosphate synthetase 1 deficiency (CPS1D) [MIM:237300]. CPS1D is an autosomal ...
Carbamoyl phosphate synthase [ammonia] mitochondrial antibody. *Carbamoyl phosphate synthase antibody. *Carbamoyl phosphate ... Carbamoyl phosphate synthetase I antibody. *Carbamoyl-phosphate synthase [ammonia] antibody. *Carbamoyl-phosphate synthetase I ... Carbamoyl phosphate synthetase 1 (CPS1) as a prognostic marker in chronic hepatitis C infection.. APMIS 127:93-105 (2019). Read ... Defects in CPS1 are the cause of carbamoyl phosphate synthetase 1 deficiency (CPS1D) [MIM:237300]. CPS1D is an autosomal ...
Carbamoyl-Phosphate Synthase (Ammonia)); ppublish] ... carbamoyl-phosphate synthetase I and ornithine carbamoyl ... ArticleTitleCrystal structure of human ornithine transcarbamylase complexed with carbamoyl phosphate and L-norvaline at 1.9 Å ... An in vitro versus in vivo study using microcarbon electrodes in neuronal nitric oxide synthase and mice brain. Neurosci Lett ... P Lirk G Hoffmann J Rieder (2002) ArticleTitleInducible nitric oxide synthase - time for reappraisal. Curr Drug Targets Inflamm ...
... carbamoyl-phosphate synthase (ammonia) [EC:6.3.4.16] K11540 CAD; carbamoyl-phosphate synthase / aspartate carbamoyltransferase ... 107748079 gmps; GMP synthase [glutamine-hydrolyzing] 107741925 LOW QUALITY PROTEIN: carbamoyl-phosphate synthase [ammonia] ... 107723076 1-acylglycerol-3-phosphate O-acyltransferase ABHD5-like 107706166 1-acylglycerol-3-phosphate O-acyltransferase ABHD5- ... 107749915 glutamine--fructose-6-phosphate aminotransferase [isomerizing] 2-like 107710818 glutamine--fructose-6-phosphate ...
"Inhibition of carbamoyl-phosphate synthase (ammonia) by Tris and Hepes. Effect on Ka for N-acetylglutamate" (PDF). Biochem. J. ... Carbamoyl phosphate synthetase catalyzes the ATP-dependent synthesis of carbamoyl phosphate from glutamine (EC 6.3.5.5) or ... Carbamoyl phosphate synthetase I (mitochondria, urea cycle). *Carbamoyl phosphate synthetase II (cytosol, pyrimidine metabolism ... Carboxy phosphate reacts with ammonia to give carbamic acid. In turn, carbamic acid reacts with a second ATP to give carbamoyl ...
... carbamoyl-phosphate synthase (ammonia) [EC:6.3.4.16] K00820 glmS; glucosamine---fructose-6-phosphate aminotransferase ( ... 100760467 Cps1; carbamoyl-phosphate synthase 1 100767191 Gfpt2; glutamine-fructose-6-phosphate transaminase 2 103159270 ... carbamoyl-phosphate synthase (ammonia) [EC:6.3.4.16] K14454 GOT1; aspartate aminotransferase, cytoplasmic [EC:2.6.1.1] K14455 ... 100760467 Cps1; carbamoyl-phosphate synthase 1 100762297 Got1; glutamic-oxaloacetic transaminase 1 100762838 Got2; glutamic- ...
Next-day shipping cDNA ORF clones derived from Cps1 carbamoyl-phosphate synthetase 1 available at GenScript, starting from $ ... carbamoyl-phosphate synthetase 1. Names. carbamoyl-phosphate synthase [ammonia], mitochondrial. CPSase I. carbamoyl-phosphate ... carbamoyl-phosphate synthase [ammonia], mitochondrial isoform X1. NM_001080809.2. NP_001074278.1. carbamoyl-phosphate synthase ... carbamoyl-phosphate synthase [ammonia], mitochondrial isoform X1. Comment. Comment: MODEL REFSEQ: This record is predicted by ...
Carbamoyl-phosphate synthase [ammonia], mitochondrial Aliases:. Not Available. RefSeq:. Not Available. Ensembl:. ... catalyzes synthesis of carbamyl phosphate from NH3, HCO3, and 2ATP [RGD, Feb 2006] ...
carbamoyl-phosphate synthase (ammonia);. carbon-dioxide---ammonia ligase;. carbamoylphosphate synthase;. carbamylphosphate ... carbamoylphosphate synthase (ammonia);. carbamoylphosphate synthetase;. carbamylphosphate synthetase I;. CPSI (gene name);. ... phosphate [CPD:C00009];. carbamoyl phosphate [CPD:C00169];. carboxyphosphate [CPD:C20969];. carbamate [CPD:C01563]. ... 2 ATP + NH3 + hydrogencarbonate = 2 ADP + phosphate + carbamoyl phosphate (overall reaction) [RN:R00149];. (1a) ATP + ...
6.3.4.16 carbamoyl-phosphate synthase (ammonia) 6.3.4.17 formate---dihydrofolate ligase 6.3.4.18 5-(carboxyamino)imidazole ...
Methylglyoxal synthase-like. 1. INHERITED FROM: Carbamoyl-phosphate synthase (ammonia). Carbamoyl phosphate synthetase, large ... INHERITED FROM: Carbamoyl-phosphate synthase (ammonia). 52021,52317. *52021 - Carbamoyl phosphate synthetase, small subunit N- ... INHERITED FROM: Carbamoyl-phosphate synthase (ammonia). 52021,52317,52440. *52021 - Carbamoyl phosphate synthetase, small ... INHERITED FROM: Carbamoyl-phosphate synthase (ammonia). 48108,52440,56059. *48108 - Carbamoyl phosphate synthetase, large ...
Mutations in this gene have been associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent ... The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. This ... The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. This ... CPS1 carbamoyl-phosphate synthase 1. Also known as: PHN; CPSASE1*See all available tests in GTR for this gene ...
Carbamoyl-Phosphate Synthase (Ammonia) * Hydranencephaly * Decidua * Lymphoma * Murine Leukemia Viruses * Leukemia * DNA ...
... carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity, cellular response to amino acid stimulus, glutamate metabolic ... Carbamoyl-phosphate synthetase ammonia chainUniRule annotation. Manual assertion according to rulesi ... Carbamoyl-phosphate synthase large chain (carB), Carbamoyl-phosphate synthase small chain (carA) ... Carbamoyl-phosphate synthase large chain (carB), Carbamoyl-phosphate synthase small chain (carA) ...
Gain of glutaminase function in mutants of the ammonia-specific frog carbamoyl phosphate synthetase. Saeed-Kothe, A., Powers- ... Specificity determining residues in ammonia- and glutamine-dependent carbamoyl phosphate synthetases. Saeed-Kothe, A., Powers- ... Structure of a bacterial pyridoxal 5-phosphate synthase complex. Strohmeier, M., Raschle, T., Mazurkiewicz, J., Rippe, K., ... We predict a structure of the glutamine amidotransferase subunit (hisH) of imidazole glycerol phosphate synthase (IGPS) which ...
Charles R, De GA, Lamers WH, Moorman AF: Control of the changes in rat-liver carbamoyl-phosphate synthase (ammonia) protein ... Expression of the genes of the ATP synthase complex, ATP synthase subunit α (Atp5a1), ATP synthase δ chain (Atp5d) and ATP ... Blood ammonia levels were determined immediately after collecting blood from the caval vein, using Ammonia Checker II (model AA ... The coordinate control of ammonia detoxification and the source of ammonia during prolonged fasting therefore deserve attention ...
Carbamoyl-phosphate synthase (ammonia) (substance). Code System Preferred Concept Name. Carbamoyl-phosphate synthase (ammonia ... Carbamoyl phosphate synthetase 1 Current Synonym true false 71077016 Carbamoyl-phosphate synthase (ammonia) Current Synonym ...
Carbamoyl-Phosphate Synthase (Ammonia) Strategic investment explains patterns of cooperation and cheating in a microbe. ...
Deconstruction of the catalytic array within the amidotransferase subunit of carbamoyl phosphate synthetase. ... Carbamoyl-phosphate synthase [ammonia], mitochondrial,Carbamoyl-phosphate synthetase I,Cps1,CPSase I,Mouse,Mus musculus. ... Carbamoyl-phosphate synthase [ammonia], mitochondrial,Carbamoyl-phosphate synthetase I,Cps1,CPSase I,Rat,Rattus norvegicus. ... Carbamoyl-phosphate synthase [ammonia], mitochondrial,Carbamoyl-phosphate synthetase I,CPS1,CPSase I,Homo sapiens,Human. ...
Ammonia is converted to carbamoyl phosphate by the enzyme carbamoyl phosphate synthase, and then enters the urea cycle to be ... Laboratory use of ammonia solutions. The hazards of ammonia solutions depend on the concentration: "dilute" ammonia solutions ... ammonia solution, AgBr is only soluble in concentrated ammonia solution while AgI is insoluble in aqueous solution of ammonia. ... Ammonia can act as a ligand in transition metal complexes. For historical reasons, ammonia is named ammine in the nomenclature ...
Carbamoyl-Phosphate Synthase (Ammonia) / genetics Actions. * Search in PubMed * Search in MeSH ... The lead variant on 2q24 (rs715) localizes to carbamoyl-phosphate synthase 1 (CPS1), which encodes a mitochondrial enzyme that ... through the rate-limiting reaction catalysed by carbamoyl-phosphate synthase 1 (CPS1), or can be converted back to glycine ... The T1405N carbamoyl phosphate synthetase polymorphism does not affect plasma arginine concentrations in preterm infants. ...
Carbamoyl-Phosphate Synthase (Ammonia) / genetics* Actions. * Search in PubMed * Search in MeSH ...
Carbamoyl-Phosphate Synthase I Deficiency Disease * Carbamoyl-Phosphate Synthase (Ammonia) * 3-Methylglutaconic Aciduria ... 3-Methylglutaconic aciduria, a frequent but underrecognized finding in carbamoyl phosphate synthetase I deficiency. Rokicki, D ...
Carbamoyl-phosphate synthase large chain (carB) Recombinant Protein-NP_705989.1 (MBS1010922) product datasheet at MyBioSource, ... Carbamoyl-phosphate synthase large chain UniProt Synonym Protein Names Carbamoyl-phosphate synthetase ammonia chain. ... Carbamoyl-phosphate synthase large chain (carB), Recombinant Protein. Also Known As Recombinant Shigella flexneri Carbamoyl- ... carbamoyl phosphate synthase large subunit UniProt Protein Name ... carbamoyl phosphate synthase large subunit NCBI Official Symbol ...
  • A gene on chromosome 2q35 that encodes a mitochondrial enzyme which catalyses synthesis of carbamoyl phosphate from ammonia and bicarbonate, a reaction that is the first committed step of the urea cycle and important in removing excess ammonia from cells. (thefreedictionary.com)
  • However, despite the important role of NAGS in ammonia removal, little is known about the mechanisms of its regulation. (nih.gov)
  • Studies suggest that the underlying cause of the hyperammonemia is the inhibition of N -acetylglutamate synthase (NAGS) activity by free propionic acid. (medscape.com)
  • For the treatment of acute and chronic hyperammonaemia in patients with N-acetylglutamate synthase (NAGS) deficiency. (drugbank.ca)
  • Carbaglu® is indicated as an adjunctive therapy in pediatric and adult patients for the treatment of acute hyperammonemia due to the deficiency of the hepatic enzyme N-acetylglutamate synthase (NAGS). (rxlist.com)
  • The NAGS gene provides instructions for making the enzyme N-acetylglutamate synthase. (medlineplus.gov)
  • 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)
  • 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)
  • Researchers in the region are focusing on adjunctive treatment and maintenance therapy associated with acute hyperammonemia and chronic hyperammonemia, which are caused by deficiency of N-acetylglutamate synthase (NAGS) in elder population and pediatric patients. (prsync.com)
  • The biochemical urea cycle pathway involves 5 catalytic enzmes and a cofactor producing enzyme: N-acetylglutamate synthase (NAGS), as well as 2 transporters: ornithase translocase and citrin. (carbaglu.net)
  • Caldovic L, Morizono H, Tuchman M. Mutations and polymorphisms in the human N-acetylglutamate synthase (NAGS) gene. (carbaglu.net)
  • Carbamoyl phosphate synthase (CPSase) is a heterodimeric enzyme composed of a small and a large subunit (with the exception of CPSase III, which is composed of a single polypeptide that may have arisen from gene fusion of the glutaminase and synthetase domains ). (wikipedia.org)
  • [7] The carboxy phosphate domain found duplicated in the large subunit of CPSase is also present as a single copy in the biotin -dependent enzymes acetyl-CoA carboxylase (ACC), propionyl-CoA carboxylase (PCCase), pyruvate carboxylase (PC) and urea carboxylase . (wikipedia.org)
  • The large subunit in bacterial CPSase has four structural domains: the carboxy phosphate domain 1, the oligomerisation domain, the carbamoyl phosphate domain 2 and the allosteric domain. (wikipedia.org)
  • Carbamoyl phosphate synthase (CPSase) is a heterodimeric enzyme composed of a small and a large subunit (with the exception of CPSase III, see below). (ebi.ac.uk)
  • This entry represents the ATP-binding domain found in the large subunit of carbamoyl phosphate synthase, as well as in other proteins, including acetyl-CoA carboxylases and pyruvate carboxylases. (ebi.ac.uk)
  • The large subunit contains two active sites, one for the production of carboxyphosphate, and the other for the production of carbamoyl phosphate. (omicsgroup.org)
  • [ 1 ] Connecting the two subunits is a tunnel of sorts, which directs the ammonia from the small subunit to the large subunit. (omicsgroup.org)
  • The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. (nih.gov)
  • Carbamoyl phosphate synthetase catalyzes the ATP-dependent synthesis of carbamoyl phosphate from glutamine ( EC 6.3.5.5 ) or ammonia ( EC 6.3.4.16 ) and bicarbonate. (wikipedia.org)
  • A gene on chromosome 2q35 that encodes a mitochondrial enzyme which catalyses synthesis of carbamoyl phosphate from ammonia and bicarbonate, a reaction that is the first committed step of the urea cycle and important in removing excess ammonia from cells. (thefreedictionary.com)
  • CPSase catalyses the synthesis of carbamoyl phosphate from biocarbonate, ATP and glutamine ( EC:6.3.5.5 ) or ammonia ( EC:6.3.4.16 ), and represents the first committed step in pyrimidine and arginine biosynthesis in prokaryotes and eukaryotes, and in the urea cycle in most terrestrial vertebrates [ PMID: 10387030 , PMID: 11212301 ]. (ebi.ac.uk)
  • This provides the ammonium ion used in the initial synthesis of carbamoyl phosphate. (wikipedia.org)
  • [1] This enzyme catalyzes the reaction of ATP and bicarbonate to produce carboxy phosphate and ADP . (wikipedia.org)
  • The encoded protein plays a role in the detoxification of ammonia by catalyzing the first step in the urea cycle in which carbomyl-phosphate is synthesized from ammonia and bicarbonate. (genscript.com)
  • Carbamoyl phosphate synthetase from Escherichia coli catalyzes the formation of carbamoyl phosphate from bicarbonate, glutamine, and two molecules of ATP. (antibody-antibodies.com)
  • Each subdomain independently binds to ATP and it is suggested that the two homologous halves act separately, one to catalyze the phosphorylation of bicarbonate to carboxy phosphate and the other that of carbamate to carbamyl phosphate. (expasy.org)
  • Carbamoyl Phosphate Synthetase I (CPSI) transfers an ammonia molecule from glutamine or glutamate to a molecule of bicarbonate that has been phosphorylated by a molecule of ATP. (omicsgroup.org)
  • Once ammonia has been brought into the mitochondria via glutamine or glutamate, it is CPSI's job to add the ammonia to bicarbonate along with a phosphate group to form carbamoyl phosphate. (omicsgroup.org)
  • Formation of carbamoyl phosphate: Aspartate 1 F. Oraby's illustrated reviews of biochemistry 20 B. Carbon atom: Protein metabolism 21 N. (homeranking.info)
  • a phosphotransferase catalyzing the formation of carbamoyl phosphate. (thefreedictionary.com)
  • Its synthesis is initiated by the formation of carbamoyl phosphate (CP) in the cytoplasm, with ammonia derived from glutamine. (biomedsearch.com)
  • Ammonia is a chemical compound of nitrogen and hydrogen , with the formula NH 3 . (newworldencyclopedia.org)
  • In living systems, ammonia plays several important roles, serving as a source of nitrogen for plants and helping maintain acid/base balance in animals. (newworldencyclopedia.org)
  • In 1909, Fritz Haber and Carl Bosch developed a method of producing ammonia from atmospheric nitrogen. (newworldencyclopedia.org)
  • Patients with carbamoyl-phosphate synthase-1 deficiency lack 'carbamoyl-phosphate synthase', one of the liver enzymes that are needed to get rid of excess nitrogen. (europa.eu)
  • In the absence of this enzyme, excess nitrogen accumulates in the body in the form of ammonia, which can be toxic at high levels, especially to the brain. (europa.eu)
  • In addition, patients were advised to control their dietary intake of proteins, which are rich in nitrogen, to reduce the amount of ammonia formed in the body. (europa.eu)
  • Flow of nitrogen from ambto acid Formation of citrulline: Carbamoyl phosphate synthase II is a cytosolic enzyme used for pyrimidine synthesis. (homeranking.info)
  • There is a third form of the enzyme, CPSase III, found in fish, which uses glutamine as a nitrogen source instead of ammonia [ PMID: 17451989 ]. (ebi.ac.uk)
  • This allows the levels of nitrogen in the body to decrease, reducing the amount of ammonia produced. (europa.eu)
  • Carbamoyl phosphate synthetase II is a cytosolic enzyme that, under physiological conditions, uses l -glutamine as the nitrogen source (producing l -glutamate) instead of NH 3 , is not activated by N -acetylglutamate, and participates in pyrimidine biosynthesis. (thefreedictionary.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)
  • 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)
  • As a result, excess nitrogen is not converted to urea for removal, and ammonia accumulates in the blood. (medlineplus.gov)
  • Organisms that cannot easily and safely remove nitrogen as ammonia convert it to a less toxic substance, such as urea, via the urea cycle, which occurs mainly in the liver. (wikipedia.org)
  • The urea cycle is essential to these organisms, because if the nitrogen or ammonia are not eliminated from the organism it can be very detrimental. (wikipedia.org)
  • Sometimes the body does not produce enough CPSI due to a mutation in the genetic code, resulting in poor metabolism of proteins and nitrogen, as well as high levels of ammonia in the body. (omicsgroup.org)
  • Excreting the excess nitrogen prevents it from accumulating in the form of ammonia, which is toxic. (nih.gov)
  • The specific role of carbamoyl phosphate synthetase I is to control the first step of the urea cycle, a reaction in which excess nitrogen compounds are incorporated into the cycle to be processed. (nih.gov)
  • The nitrogen atom in the molecule has a lone electron pair, and ammonia acts as a base, a proton acceptor. (bionity.com)
  • The entire nitrogen content of all manufactured organic compounds is derived from ammonia. (bionity.com)
  • When his body can't remove nitrogen, ammonia forms and collects in his blood . (webmd.com)
  • It catalyzes the condensation and activation of NH 4 + and HCO 3 - to form carbamoyl phosphate, the first of the cycle's two nitrogen-containing substrates, with the concomitant hydrolysis of two ATPs. (conservapedia.com)
  • Mitochondrial CPS I uses ammonia as its nitrogen donor and participates in urea biosynthesis. (conservapedia.com)
  • Under these conditions, the genes necessary for conversion of L-glutamine to carbamoyl-P, conversion of PRPP to 5-phospho-ribosyl-amine, conversion of 5-phospho-ribosyl-amine until to IMP, cyclization of carbamoyl-aspartate to dihydroorotate and its conversion to orotate, conversion of serine to 5, 10-tetrahydrofolate are not expressed. (unam.mx)
  • Less detailed: The presence of hypoxanthine inhibits the expression of genes needful for conversion of L-glutamine to carbamoyl-P, conversion of PRPP to 5-phospho-ribosyl-amine, conversion of 5-phospho-ribosyl-amine until to IMP, cyclization of carbamoyl-aspartate to dihydroorotate and its conversion to orotate, conversion of serine to 5, 10-tetrahydrofolate. (unam.mx)
  • This gene encodes a trifunctional protein which is associated with the enzymatic activities of the first 3 enzymes in the 6-step pathway of pyrimidine biosynthesis: carbamoylphosphate synthetase (CPS II), aspartate transcarbamoylase, and dihydroorotase. (thermofisher.com)
  • The reactions of the urea cycle 1 L-ornithine 2 carbamoyl phosphate 3 L-citrulline 4 argininosuccinate 5 fumarate 6 L-arginine 7 urea L-Asp L-aspartate CPS-1 carbamoyl phosphate synthetase I OTC Ornithine transcarbamoylase ASS argininosuccinate synthetase ASL argininosuccinate lyase ARG1 arginase 1 Before the urea cycle begins ammonia is converted to carbamoyl phosphate. (wikipedia.org)
  • We can summarize this by combining the reactions: CO2 + glutamate + aspartate + 3 ATP + 2 NAD++ 3 H2O → urea + α-ketoglutarate + oxaloacetate + 2 ADP + 2 Pi + AMP + PPi + 2 NADH The two NADH produced can provide energy for the formation of 5 ATP (cytosolic NADH provides 2.5 ATP with the malate-aspartate shuttle in human liver cell), a net production of two high-energy phosphate bond for the urea cycle. (wikipedia.org)
  • GENTAUR antibody-antibodies.com The Marketplace for Antibodies : Deconstruction of the catalytic array within the amidotransferase subunit of carbamoyl phosphate synthetase. (antibody-antibodies.com)
  • The structure of carbamoyl phosphate synthetase determined to 2.1 A resolution. (ebi.ac.uk)
  • Co‑expression of the carbamoyl‑phosphate synthase 1 gene and its long non‑coding RNA correlates with poor prognosis of patients with intrahepatic cholangiocarcinoma. (nih.gov)
  • A small chain (gene carA) that provides glutamine amidotransferase activity (GATase) necessary for removal of the ammonia group from glutamine, and a large chain (gene carB) that provides CPSase activity. (expasy.org)
  • This study examined the spatiotemporal patterns of gene expression of four major O-UC enzymes: carbamoyl phosphate synthase III (CPSIII), ornithine transcarboxylase, arginosuccinate synthetase, and arginosuccinate lyase, using real-time PCR and whole mount in situ hybridization. (nih.gov)
  • The CBS enzyme is located on the long arm of chromosome 21 (21q22.3) and the mutation of the CBS gene may result in reduced activity of cystathionine beta-synthase resulting in classical homocystinuria. (medicalbiochemist.com)
  • Title: Protein tyrosine nitration of mitochondrial carbamoyl phosphate synthetase 1 and its functional consequences. (genscript.com)
  • Molecular defects in human carbamoyl phosphate synthetase I: mutational spectrum, diagnostic and protein structure considerations. (expasy.org)
  • During acute hyperammonemic episodes concomitant administration of Carbaglu® with other ammonia lowering therapies such as alternate pathway medications, hemodialysis , and dietary protein restriction are recommended. (rxlist.com)
  • During maintenance therapy, the concomitant use of other ammonia lowering therapies and protein restriction may be reduced or discontinued based on plasma ammonia levels. (rxlist.com)
  • Proteins in NPC: (1) O35828 Coronin-7, (2) P16599 Tumor necrosis factor, (3) P34058 Heat shock protein HSP 90-beta, (4) Q63264 Interleukin-1 beta, (5) Q63921 Prostaglandin G/H synthase 1, (6) Q64244 ADP-ribosyl cyclase 1. (bioz.com)
  • Proteins in HC: (1) P07756 Carbamoyl-phosphate synthase [ammonia], (2) P08932 T-kininogen 2, (3) P19488 UDP-glucuronosyltransferase 2B37, (4) Q6DGG0 Peptidyl-prolyl cis-trans isomerase D. Part B) demonstrates the distribution of distinct proteins within the three fractions, supernatant, cytoplasm and nuclear protein fractions, underneath the respective treatment of the cells, which gives an overview of the responsiveness of the cells. (bioz.com)
  • Ravicti is indicated for use as adjunctive therapy for chronic management of adult and paediatric patients ≥2 months of age with urea cycle disorders (UCDs) including deficiencies of carbamoyl phosphate-synthase-I (CPS), ornithine carbamoyltransferase (OTC), argininosuccinate synthetase (ASS), argininosuccinate lyase (ASL), arginase I (ARG) and ornithine translocase deficiency hyperornithinaemia-hyperammonaemia homocitrullinuria syndrome (HHH) who cannot be managed by dietary protein restriction and/or amino acid supplementation alone. (europa.eu)
  • It is also elevated by ammonia intoxication and during feeding of diets high in protein, high in lysine with respect to arginine, or deficient in arginine, ornithine, and citrulline. (biomedsearch.com)
  • The amidotransferase family of enzymes: molecular machines for the production and delivery of ammonia. (ebi.ac.uk)
  • In the first step, catalyzed by glutamine-PRPP amidotransferase, glutamine acts as a source of ammonia and PPi (inorganic pyrophosphate) is released. (wikibooks.org)
  • Carbamoyl Phosphate Synthetase I is a ligase enzyme located in the mitochondria involved in the production of urea. (omicsgroup.org)
  • however, the N-terminal carboxy phosphate domain catalyses the phosphorylation of biocarbonate, while the C-terminal domain catalyses the phosphorylation of the carbamate intermediate . (wikipedia.org)
  • [8] CPSase heterodimers from Escherichia coli contain two molecular tunnels: an ammonia tunnel and a carbamate tunnel. (wikipedia.org)
  • These inter-domain tunnels connect the three distinct active sites, and function as conduits for the transport of unstable reaction intermediates (ammonia and carbamate) between successive active sites . (wikipedia.org)
  • and the phosphorylation of carbamate forming carbamoyl phosphate. (kegg.jp)
  • Structural defects within the carbamate tunnel of carbamoyl phosphate synthetase. (ebi.ac.uk)
  • Carbamoyl phosphate synthetase I is the rate-limiting enzyme that catalyzes the first committed step of the hepatic urea cycle. (antibody-antibodies.com)
  • Carbomoyl phosphate synthetase I is a mitochondrial enzyme that catalyzes the reaction of 2ATP, NH 3 , CO 2 , and H 2 O to carbamoyl phosphate, 2ADP, and orthophosphate. (thefreedictionary.com)
  • The enzyme Glycogen synthase catalyzes the addition of glucose molecules at the nonreducing end of core glycogen molecule In this reaction, an activated UDP-glucose molecule forms 1-4 glycosidic linkage with existing glucose moiety of glycogen molecule and free UDP is liberated. (medicalbiochemist.com)
  • The carA and carB genes code the small and large subunits of carbamoyl-phosphate synthase (CPS) that responsible for arginine and pyrimidine production. (beds.ac.uk)
  • Since N -acetylglutamate (NAG) is the allosteric activator of carbamoylphosphate synthase, the entry step into the urea cycle, decreased ureagenesis occurs with accumulation of free ammonia. (medscape.com)
  • Carglumic acid, the active substance, is a Carbamoyl Phosphate Synthetase 1 (CPS 1) activator and is soluble in boiling water, slightly soluble in cold water, practically insoluble inorganic solvents. (rxlist.com)
  • The 6th one is N- acetylglutamate that acts as allosteric activator of carbamoyl phosphate synthase I. Arginine ': Then urea is transported by the blood to. (homeranking.info)
  • Carglumic acid is a structural analogue of N-acetylglutamate, which is the naturally occurring activator of carbamoyl phosphate synthetase, the first enzyme of the urea cycle. (medscape.com)
  • Your search returned 15 carbamoyl-phosphate synthase 1 ELISA ELISA Kit across 4 suppliers. (biocompare.com)
  • The urea cycle converts toxic ammonia to urea within the liver of mammals. (nih.gov)
  • Title: Isoaspartate, carbamoyl phosphate synthase-1, and carbonic anhydrase-III as biomarkers of liver injury. (genscript.com)
  • Title: Carbamoyl phosphate synthetase-1 is a rapid turnover biomarker in mouse and human acute liver injury. (genscript.com)
  • On 17 December 2010, orphan designation (EU/3/10/821) was granted by the European Commission to Cytonet GmbH & Co. KG, Germany, for human heterologous liver cells (for infusion) for the treatment of carbamoyl-phosphate synthase-1 deficiency. (europa.eu)
  • The sponsor has provided sufficient information to show that human heterologous liver cells (for infusion) might be of significant benefit for patients with carbamoyl-phosphate synthase-1 deficiency because it works in a different way to existing treatment and early studies indicate that it might improve the treatment of patients with this condition. (europa.eu)
  • When the medicine is injected repeatedly into the portal vein (the vein leading to the liver) of patients with carbamoyl-phosphate synthase-1 deficiency, some of the liver cells it contains are expected to settle in the recipient's liver and start producing the missing liver enzyme, thus helping to alleviate the symptoms of the disease. (europa.eu)
  • Urea formation is the pathway through which the liver can convert toxic ammonia into non-toxic urea. (homeranking.info)
  • Liver cell failure: The diseased liver cells cannot convert ammonia into urea. (homeranking.info)
  • Analysis of in vivo models have showcased carglumic acid to stimulate liver carbamoyl phosphate synthetase. (prsync.com)
  • Urea is produced primarily in the liver, which acts as the main ammonia detoxification center. (carbaglu.net)
  • Dosing should be titrated based on individual patient plasma ammonia levels and clinical symptoms. (rxlist.com)
  • The investigators will realize this goal by randomizing each hyperammonemic episode from every subject to NCG (NCG)+standard treatment (NCG-STD) versus placebo+standard treatment (PLBO-STD) and subsequently gauging response with the primary outcome of plasma ammonia levels, in addition to the plasma glutamine, the Functional Status Scale, and the length of hospitalization. (clinicaltrials.gov)
  • Ammonia contributes significantly to the nutritional needs of the planet as a precursor to foodstuffs and fertilizers. (bionity.com)
  • In all organisms, carbamoylphosphate (CP) is a precursor common to the synthesis of arginine and pyrimidines. (springer.com)
  • Isovaleric aciduria (IVA), propionic aciduria (PA) and methylmalonic aciduria (MMA) are inherited organic acidurias (OAs) in which impaired organic acid metabolism induces hyperammonaemia arising partly from secondary deficiency of N-acetylglutamate (NAG) synthase. (biomedcentral.com)
  • Carbamoylphosphate is a common intermediate in the metabolic pathways leading to the biosynthesis of arginine and pyrimidines. (nih.gov)
  • It is also produced from L-arginine as a by-product of the reaction catalyzed by the enzyme NO synthase. (herbal-ingredients.com)
  • The expression of carbamoyl phosphate synthetase is controlled by various metabolites along the pathways for pyrimidine and arginine synthesis. (beds.ac.uk)
  • In Escherichia coli and most other Gram-negative bacteria, CP is produced by a single enzyme, carbamoylphosphate synthase (CPSase), encoded by the carAB operon. (springer.com)
  • Chemically carglumic acid is N-carbamoyl-L-glutamic acid or (2S)-2-(carbamoylamino) pentanedioic acid, with a molecular weight of 190.16. (rxlist.com)
  • The primary outcome was change in plasma ammonia from baseline to endpoint (last available ammonia measurement at ≤18 hours after the last carglumic acid administration, or on Day 15) for each episode. (biomedcentral.com)
  • Although patients receiving carglumic acid with scavengers had a greater reduction in plasma ammonia, the endpoint ammonia levels were similar with or without scavenger therapy. (biomedcentral.com)
  • Carglumic acid when used with or without ammonia scavengers, is an effective treatment for restoration of normal plasma ammonia concentrations in hyperammonaemic episodes in OA patients. (biomedcentral.com)
  • Well known as N-carbamoyl-L-glutamic acid (NCGA) or Carbaglu, carglumic acid stimulates the initial enzyme in urea cycle, which helps in detoxification and removal of ammonia from blood cells. (prsync.com)
  • Although carbamoyl phosphate synthetase has a relatively lower affinity for carglumic acid than N-acetylglutamate (NAG), carglumic acid has been represented in vitro to activate carbamoyl phosphate synthetase, being much more effective in preventing ammonia intoxication. (prsync.com)
  • Carglumic acid is further being utilized for application in enhancing ammonia detoxification in patients with organic acidurias. (prsync.com)
  • A second molecule of ATP then phosphorylates carbamic acid, creating carbamoyl phosphate. (wikipedia.org)
  • The resulting molecule of carbamoyl phosphate leaves the enzyme. (omicsgroup.org)
  • This ATP-binding domain coupling works in a way such that a molecule of ATP binding at one site (domain C) conformationally allows synthesis at the other domain (domain B). If this is the case, carbamoyl phosphate is, in fact, not formed in step 5 (of the included mechanism below) by ejecting ADP but rather in step 4 by protonating the alcohol group and then kicking it off as water. (omicsgroup.org)
  • The ammonia molecule has a trigonal pyramid shape, as predicted by VSEPR theory. (bionity.com)
  • This shape gives the molecule an overall dipole moment and makes it polar so that ammonia readily dissolves in water. (bionity.com)
  • 1,2) This antigen was shown to be carbamoyl phosphate synthetase 1, an enzyme involved in urea synthesis. (thefreedictionary.com)
  • When there is insufficient capacity for detoxifying the load of ammonia presented for urea synthesis, CP leaves the mitochondria and enters the pyrimidine pathway, where orotic acid biosynthesis is stimulated, orotic acid excretion in urine then increases. (biomedsearch.com)
  • N-acetylglutamate synthase controls the production of a compound called N-acetylglutamate in the mitochondria, the energy-producing centers in cells. (medlineplus.gov)
  • Sticking to the analogy of this cycle being like a waste management facility, power is received from the power plant (mitochondria), the previous cycle BH4 share their facility for the urea cycle to clean out the ammonia. (fourie.net.za)
  • Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell. (nih.gov)
  • Arginosuccinate synthetase is an enzyme in the urea cycle, which removes excess ammonia from the body. (wikipedia.org)
  • Excess ammonia is toxic to the central nervous system. (homeranking.info)
  • As early as the thirteenth century, ammonia was known to the alchemists in the form of sal ammoniac, which was mentioned by Albertus Magnus . (newworldencyclopedia.org)
  • Other mutations result in production of an abnormally short N-acetylglutamate synthase enzyme or prevent any enzyme from being produced at all. (medlineplus.gov)
  • Sancho-Vaello E, Marco-Marín C, Gougeard N, Fernández-Murga L, Rüfenacht V, Mustedanagic M, Rubio V, Häberle J. Understanding N-Acetyl-L-Glutamate Synthase Deficiency: Mutational Spectrum, Impact of Clinical Mutations on Enzyme Functionality, and Structural Considerations. (medlineplus.gov)
  • Furthermore, people in the family members encode both carbamoyl phosphate synthase (ECH_0503/ECH_0378) and bifunctional glutamate synthase subunit beta/2-polyprenylphenol hydroxylase (ECH_0778), both which can convert glutamine to ammonia and glutamate (Desk?1). (biomasswars.com)