Carbamyl phosphate. 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.

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.

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 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.

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.

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.

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.

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.

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

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 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.

Disorders affecting amino acid metabolism. The majority of these disorders are inherited and present in the neonatal period with metabolic disturbances (e.g., ACIDOSIS) and neurologic manifestations. They are present at birth, although they may not become symptomatic until later in life.

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 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.

The rate dynamics in chemical or physical systems.

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

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

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

Carbon-containing phosphoric acid derivatives. Included under this heading are compounds that have CARBON atoms bound to one or more OXYGEN atoms of the P(=O)(O)3 structure. Note that several specific classes of endogenous phosphorus-containing compounds such as NUCLEOTIDES; PHOSPHOLIPIDS; and PHOSPHOPROTEINS are listed elsewhere.

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.

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.

A mitochondrial matrix enzyme that catalyzes the synthesis of L-GLUTAMATE to N-acetyl-L-glutamate in the presence of ACETYL-COA.

Organic compounds that contain phosphorus as an integral part of the molecule. Included under this heading is broad array of synthetic compounds that are used as PESTICIDES and DRUGS.

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

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.

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

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.

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.

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)

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.

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

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

A closely related group of toxic substances elaborated by various strains of Streptomyces. They are 26-membered macrolides with lactone moieties and double bonds and inhibit various ATPases, causing uncoupling of phosphorylation from mitochondrial respiration. Used as tools in cytochemistry. Some specific oligomycins are RUTAMYCIN, peliomycin, and botrycidin (formerly venturicidin X).

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.

Organic compounds that contain two nitro groups attached to a phenol.

The interference in synthesis of an enzyme due to the elevated level of an effector substance, usually a metabolite, whose presence would cause depression of the gene responsible for enzyme synthesis.

A genus of the family Muridae having three species. The present domesticated strains were developed from individuals brought from Syria. They are widely used in biomedical research.

The sum of the weight of all the atoms in a molecule.

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)

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.

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

A genus of motile or nonmotile gram-positive bacteria of the family Clostridiaceae. Many species have been identified with some being pathogenic. They occur in water, soil, and in the intestinal tract of humans and lower animals.

Phosphoric acid esters of inositol. They include mono- and polyphosphoric acid esters, with the exception of inositol hexaphosphate which is PHYTIC ACID.

A fractionated cell extract that maintains a biological function. A subcellular fraction isolated by ultracentrifugation or other separation techniques must first be isolated so that a process can be studied free from all of the complex side reactions that occur in a cell. The cell-free system is therefore widely used in cell biology. (From Alberts et al., Molecular Biology of the Cell, 2d ed, p166)

A subfamily in the family MURIDAE, comprising the hamsters. Four of the more common genera are Cricetus, CRICETULUS; MESOCRICETUS; and PHODOPUS.

Organic compounds that generally contain an amino (-NH2) and a carboxyl (-COOH) group. Twenty alpha-amino acids are the subunits which are polymerized to form proteins.

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.

The process of cleaving a chemical compound by the addition of a molecule of water.

Adenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.

Genetically identical individuals developed from brother and sister matings which have been carried out for twenty or more generations or by parent x offspring matings carried out with certain restrictions. This also includes animals with a long history of closed colony breeding.

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.

The relationship between the chemical structure of a compound and its biological or pharmacological activity. Compounds are often classed together because they have structural characteristics in common including shape, size, stereochemical arrangement, and distribution of functional groups.

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

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

An oxidative decarboxylation process that converts GLUCOSE-6-PHOSPHATE to D-ribose-5-phosphate via 6-phosphogluconate. The pentose product is used in the biosynthesis of NUCLEIC ACIDS. The generated energy is stored in the form of NADPH. This pathway is prominent in tissues which are active in the synthesis of FATTY ACIDS and STEROIDS.

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.

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.

Compounds and molecular complexes that consist of very large numbers of atoms and are generally over 500 kDa in size. In biological systems macromolecular substances usually can be visualized using ELECTRON MICROSCOPY and are distinguished from ORGANELLES by the lack of a membrane structure.

A deoxyribonucleotide polymer that is the primary genetic material of all cells. Eukaryotic and prokaryotic organisms normally contain DNA in a double-stranded state, yet several important biological processes transiently involve single-stranded regions. DNA, which consists of a polysugar-phosphate backbone possessing projections of purines (adenine and guanine) and pyrimidines (thymine and cytosine), forms a double helix that is held together by hydrogen bonds between these purines and pyrimidines (adenine to thymine and guanine to cytosine).

An important intermediate in lipid biosynthesis and in glycolysis.

Membrane proteins that are involved in the active transport of phosphate.

This is the active form of VITAMIN B 6 serving as a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid. During transamination of amino acids, pyridoxal phosphate is transiently converted into pyridoxamine phosphate (PYRIDOXAMINE).

An aldose-ketose isomerase that catalyzes the reversible interconversion of glucose 6-phosphate and fructose 6-phosphate. In prokaryotic and eukaryotic organisms it plays an essential role in glycolytic and gluconeogenic pathways. In mammalian systems the enzyme is found in the cytoplasm and as a secreted protein. This secreted form of glucose-6-phosphate isomerase has been referred to as autocrine motility factor or neuroleukin, and acts as a cytokine which binds to the AUTOCRINE MOTILITY FACTOR RECEPTOR. Deficiency of the enzyme in humans is an autosomal recessive trait, which results in CONGENITAL NONSPHEROCYTIC HEMOLYTIC ANEMIA.

The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.

A category of nucleic acid sequences that function as units of heredity and which code for the basic instructions for the development, reproduction, and maintenance of organisms.

An enzyme that transfers acyl groups from acyl-CoA to glycerol-3-phosphate to form monoglyceride phosphates. It acts only with CoA derivatives of fatty acids of chain length above C-10. Also forms diglyceride phosphates. EC 2.3.1.15.

An amino alcohol with a long unsaturated hydrocarbon chain. Sphingosine and its derivative sphinganine are the major bases of the sphingolipids in mammals. (Dorland, 28th ed)

Any salt or ester of glycerophosphoric acid.

Proteins prepared by recombinant DNA technology.

RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.

Extrachromosomal, usually CIRCULAR DNA molecules that are self-replicating and transferable from one organism to another. They are found in a variety of bacterial, archaeal, fungal, algal, and plant species. They are used in GENETIC ENGINEERING as CLONING VECTORS.

Derivatives of PHOSPHATIDIC ACIDS that lack one of its fatty acyl chains due to its hydrolytic removal.

Proteins that bind to and are involved in the metabolism of phosphate ions.

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

Phosphatidylinositols in which one or more alcohol group of the inositol has been substituted with a phosphate group.

Ca-releasing action of beta, gamma-methylene adenosine triphosphate on fragmented sarcoplasmic reticulum. (1/122)

beta,gamma-Methylene adenosine triphosphate (AMPOPCP) has two effects on fragmented sarcoplasmic reticulum (FSR), i.e., inhibition of the rate of Ca uptake and the induction of Ca release from FSR filled with Ca. The Ca release brought about by AMPOPCP has many features in common with the mechanism of Ca-induced Ca release: i) it is inhibited by 10 mM procaine; ii) the amount of Ca release increases with increase in the extent of saturation of FSR with Ca; iii) increase of the Ca concentration in the extent of saturation of FSR with Ca; iii) increase of the Ca concentration in the medium facilitates the release of Ca. However, no facilitation of Ca release upon decrease of Mg concentration in the medium is observable. AMPOPCP and caffeine potentiate each other remarkably in their Ca-releasing action, irrespective of the kind of substrate. From the mode of action of AMPOPCP on the rate of Ca uptake, the amount of phosphorylated intermediate (EP), and the effect on Sr release, it is suggested that the state of the FSR-ATP complex is crucial for Ca-induced Ca release. (+info)

Channeling of carbamoyl phosphate to the pyrimidine and arginine biosynthetic pathways in the deep sea hyperthermophilic archaeon Pyrococcus abyssi. (2/122)

The kinetics of the coupled reactions between carbamoyl-phosphate synthetase (CPSase) and both aspartate transcarbamoylase (ATCase) and ornithine transcarbamoylase (OTCase) from the deep sea hyperthermophilic archaeon Pyrococcus abyssi demonstrate the existence of carbamoyl phosphate channeling in both the pyrimidine and arginine biosynthetic pathways. Isotopic dilution experiments and coupled reaction kinetics analyzed within the context of the formalism proposed by Ovadi et al. (Ovadi, J., Tompa, P., Vertessy, B., Orosz, F., Keleti, T., and Welch, G. R. (1989) Biochem. J. 257, 187-190) are consistent with a partial channeling of the intermediate at 37 degrees C, but channeling efficiency increases dramatically at elevated temperatures. There is no preferential partitioning of carbamoyl phosphate between the arginine and pyrimidine biosynthetic pathways. Gel filtration chromatography at high and low temperature and in the presence and absence of substrates did not reveal stable complexes between P. abyssi CPSase and either ATCase or OTCase. Thus, channeling must occur during the dynamic association of coupled enzymes pairs. The interaction of CPSase-ATCase was further demonstrated by the unexpectedly weak inhibition of the coupled reaction by the bisubstrate analog, N-(phosphonacetyl)-L-aspartate (PALA). The anomalous effect of PALA suggests that, in the coupled reaction, the effective concentration of carbamoyl phosphate in the vicinity of the ATCase active site is 96-fold higher than the concentration in the bulk phase. Channeling probably plays an essential role in protecting this very unstable intermediate of metabolic pathways performing at extreme temperatures. (+info)

Carbamate kinase: New structural machinery for making carbamoyl phosphate, the common precursor of pyrimidines and arginine. (3/122)

The enzymes carbamoyl phosphate synthetase (CPS) and carbamate kinase (CK) make carbamoyl phosphate in the same way: by ATP-phosphorylation of carbamate. The carbamate used by CK is made chemically, whereas CPS itself synthesizes its own carbamate in a process involving the phosphorylation of bicarbonate. Bicarbonate and carbamate are analogs and the phosphorylations are carried out by homologous 40 kDa regions of the 120 kDa CPS polypeptide. CK can also phosphorylate bicarbonate and is a homodimer of a 33 kDa subunit that was believed to resemble the 40 kDa regions of CPS. Such belief is disproven now by the CK structure reported here. The structure does not conform to the biotin carboxylase fold found in the 40 kDa regions of CPS, and presents a new type of fold possibly shared by homologous acylphosphate-making enzymes. A molecular 16-stranded open beta-sheet surrounded by alpha-helices is the hallmark of the CK dimer. Each subunit also contains two smaller sheets and a large crevice found at the location expected for the active center. Intersubunit interactions are very large and involve a central hydrophobic patch and more hydrophilic peripheral contacts. The crevice holds a sulfate that may occupy the site of an ATP phosphate, and is lined by conserved residues. Site-directed mutations tested at two of these residues inactivate the enzyme. These findings support active site location in the crevice. The orientation of the crevices in the dimer precludes their physical cooperation in the catalytic process. Such cooperation is not needed in the CK reaction but is a requirement of the mechanism of CPSs. (+info)

The carbamoyl-phosphate synthetase of Pyrococcus furiosus is enzymologically and structurally a carbamate kinase. (4/122)

The hyperthermophiles Pyrococcus furiosus and Pyrococcus abyssi make pyrimidines and arginine from carbamoyl phosphate (CP) synthesized by an enzyme that differs from other carbamoyl-phosphate synthetases and that resembles carbamate kinase (CK) in polypeptide mass, amino acid sequence, and oligomeric organization. This enzyme was reported to use ammonia, bicarbonate, and two ATP molecules as carbamoyl-phosphate synthetases to make CP and to exhibit bicarbonatedependent ATPase activity. We have reexamined these findings using the enzyme of P. furiosus expressed in Escherichia coli from the corresponding gene cloned in a plasmid. We show that the enzyme uses chemically made carbamate rather than ammonia and bicarbonate and catalyzes a reaction with the stoichiometry and equilibrium that are typical for CK. Furthermore, the enzyme catalyzes actively full reversion of the CK reaction and exhibits little bicarbonate-dependent ATPase. In addition, it cross-reacts with antibodies raised against CK from Enterococcus faecium, and its three-dimensional structure, judged by x-ray crystallography of enzyme crystals, is very similar to that of CK. Thus, the enzyme is, in all respects other than its function in vivo, a CK. Because in other organisms the function of CK is to make ATP from ADP and CP derived from arginine catabolism, this is the first example of using CK for making rather than using CP. The reasons for this use and the adaptation of the enzyme to this new function are discussed. (+info)

Half of Saccharomyces cerevisiae carbamoyl phosphate synthetase produces and channels carbamoyl phosphate to the fused aspartate transcarbamoylase domain. (5/122)

The first two steps of the de novo pyrimidine biosynthetic pathway in Saccharomyces cerevisiae are catalyzed by a 240-kDa bifunctional protein encoded by the ura2 locus. Although the constituent enzymes, carbamoyl phosphate synthetase (CPSase) and aspartate transcarbamoylase (ATCase) function independently, there are interdomain interactions uniquely associated with the multifunctional protein. Both CPSase and ATCase are feedback inhibited by UTP. Moreover, the intermediate carbamoyl phosphate is channeled from the CPSase domain where it is synthesized to the ATCase domain where it is used in the synthesis of carbamoyl aspartate. To better understand these processes, a recombinant plasmid was constructed that encoded a protein lacking the amidotransferase domain and the amino half of the CPSase domain, a 100-kDa chain segment. The truncated complex consisted of the carboxyl half of the CPSase domain fused to the ATCase domain via the pDHO domain, an inactive dihydroorotase homologue that bridges the two functional domains in the native molecule. Not only was the "half CPSase" catalytically active, but it was regulated by UTP to the same extent as the parent molecule. In contrast, the ATCase domain was no longer sensitive to the nucleotide, suggesting that the two catalytic activities are controlled by distinct mechanisms. Most remarkably, isotope dilution and transient time measurements showed that the truncated complex channels carbamoyl phosphate. The overall CPSase-ATCase reaction is much less sensitive than the parent molecule to the ATCase bisubstrate analogue, N-phosphonacetyl-L-aspartate (PALA), providing evidence that the endogenously produced carbamoyl phosphate is sequestered and channeled to the ATCase active site. (+info)

Studies of hepatic glutamine metabolism in the perfused rat liver with (15)N-labeled glutamine. (6/122)

This study examines the role of glucagon and insulin in the incorporation of (15)N derived from (15)N-labeled glutamine into aspartate, citrulline and, thereby, [(15)N]urea isotopomers. Rat livers were perfused, in the nonrecirculating mode, with 0.3 mM NH(4)Cl and either 2-(15)N- or 5-(15)N-labeled glutamine (1 mM). The isotopic enrichment of the two nitrogenous precursor pools (ammonia and aspartate) involved in urea synthesis as well as the production of [(15)N]urea isotopomers were determined using gas chromatography-mass spectrometry. This information was used to examine the hypothesis that 5-N of glutamine is directly channeled to carbamyl phosphate (CP) synthesis. The results indicate that the predominant metabolic fate of [2-(15)N] and [5-(15)N]glutamine is incorporation into urea. Glucagon significantly stimulated the uptake of (15)N-labeled glutamine and its metabolism via phosphate-dependent glutaminase (PDG) to form U(m+1) and U(m+2) (urea containing one or two atoms of (15)N). However, insulin had little effect compared with control. The [5-(15)N]glutamine primarily entered into urea via ammonia incorporation into CP, whereas the [2-(15)N]glutamine was predominantly incorporated via aspartate. This is evident from the relative enrichments of aspartate and of citrulline generated from each substrate. Furthermore, the data indicate that the (15)NH(3) that was generated in the mitochondria by either PDG (from 5-(15)N) or glutamate dehydrogenase (from 2-(15)N) enjoys the same partition between incorporation into CP or exit from the mitochondria. Thus, there is no evidence for preferential access for ammonia that arises by the action of PDG to carbamyl-phosphate synthetase. To the contrary, we provide strong evidence that such ammonia is metabolized without any such metabolic channeling. The glucagon-induced increase in [(15)N]urea synthesis was associated with a significant elevation in hepatic N-acetylglutamate concentration. Therefore, the hormonal regulation of [(15)N]urea isotopomer production depends upon the coordinate action of the mitochondrial PDG pathway and the synthesis of N-acetylglutamate (an obligatory activator of CP). The current study may provide the theoretical and methodological foundations for in vivo investigations of the relationship between the hepatic urea cycle enzyme activities, the flux of (15)N-labeled glutamine into the urea cycle, and the production of urea isotopomers. (+info)

Substitutions in the aspartate transcarbamoylase domain of hamster CAD disrupt oligomeric structure. (7/122)

Aspartate transcarbamoylase (ATCase; EC 2.1.3.2) is one of three enzymatic domains of CAD, a protein whose native structure is usually a hexamer of identical subunits. Alanine substitutions for the ATCase residues Asp-90 and Arg-269 were generated in a bicistronic vector that encodes a 6-histidine-tagged hamster CAD. Stably transfected mammalian cells expressing high levels of CAD were easily isolated and CAD purification was simplified over previous procedures. The substitutions reduce the ATCase V(max) of the altered CADs by 11-fold and 46-fold, respectively, as well as affect the enzyme's affinity for aspartate. At 25 mM Mg(2+), these substitutions cause the oligomeric CAD to dissociate into monomers. Under the same dissociating conditions, incubating the altered CAD with the ATCase substrate carbamoyl phosphate or the bisubstrate analogue N-phosphonacetyl-L-aspartate unexpectedly leads to the reformation of hexamers. Incubation with the other ATCase substrate, aspartate, has no effect. These results demonstrate that the ATCase domain is central to hexamer formation in CAD and suggest that the ATCase reaction mechanism is ordered in the same manner as the Escherichia coli ATCase. Finally, the data indicate that the binding of carbamoyl phosphate induces conformational changes that enhance the interaction of CAD subunits. (+info)

Antitumor activity of N-(phosphonacetyl)-L-aspartic acid, a transition-state inhibitor of aspartate transcarbamylase. (8/122)

N-(Phosphonacetyl)-L-aspartate (PALA) is an analog of the transition state for the aspartate transcarbamylase reaction and has been reported previously to be a potent and specific inhibitor of de novo pyrimidine nucleotide biosynthesis. It is now shown that PALA has considerable antitumor activity against certain transplantable tumors in mice. PALA, unlike other antimetabolites, was less effective against ascitic leukemias than against two solid tumors, B16 melanoma and Lewis lung carcinoma. Another solid tumor, Ridgway osteogenic sarcoma, which is sensitivie to many established chemotherapeutic agents, did not respond to PALA. Daily or intermittent treatment with PALA did not significantly increase the life-span of mice bearing i.p. leukemia L1210. The survival time of mice bearing i.p. P388 leukemia was prolonged by PALA treatment by up to 64%. In a number of experiments mice bearing i.p. B16 melanoma survived 77 to 86% longer than did controls when treated with PALA (490 mg/kg) on Days 1, 5, and 9. Lewis lung carcinoma, a tumor refractory to most established antineoplastic agents, was highly sensitive to PALA. Treatment on Days 1, 5, and 9 following s.c. implantation of Lewis lung carcinoma was curative to 50% of the mice. If treatment was delayed until s.c. Lewis lung tumors had reached about 500 mg, PALA neither cured the mice nor produced significant tumor regression. However, extensive delay of tumor growth and prolongation of survival were still observed. (+info)

Jones ME, Spector L, Lipmann F (1955). "Carbamyl phosphate, the carbamyl donor in enzymatic citrulline synthesis". J. Am. Chem ... "Carbamyl phosphate synthesis in Neurospora crassa. I. Preliminary characterization of arginine-specific carbamyl phosphokinase ... Other names in common use include CKase, carbamoyl phosphokinase, and carbamyl phosphokinase. This enzyme participates in 4 ... carbamoyl phosphate The 3 substrates of this enzyme are ATP, NH3, and CO2, whereas its two products are ADP and carbamoyl ...

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

Jones, M. E.; Spector, L.; Lipmann, F. (February 1955). "Carbamyl Phosphate, the Carbamyl Donor in Enzymatic Citrulline ... She was notable for discovery of carbamoyl phosphate, a chemical substance that is key to the biosynthesis of arginine and urea ... The two continued to work on carbamoyl phosphate, identifying carbon dioxide or bicarbonate as the source for the initial ... Kresge, Nicole; Simoni, Robert D.; Hill, Robert L. (May 11, 2007). "Carbamoyl Phosphate Biosynthesis: the Work of Mary Ellen ...

https://en.wikipedia.org/wiki/Mary_Ellen_Jones_(chemist)

"Isolation and characterization of a naturally occurring cofactor of carbamyl phosphate biosynthesis". J Biol Chem. 230 (2): ... in which carbamoyl phosphate is produced. Carbamoyl Phosphate Synthase 1, abbreviated as CPS1, is activated by its natural ... Carbamoyl phosphate synthase I is an enzyme found in mitochondrial matrix and it catalyzes the very first reaction of the Urea ...

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

It triggers the production of the first urea cycle intermediate, carbamyl phosphate. CPSI is inactive when N-acetylglutamic ... In contrast, N-acetylglutamic acid is not the allosteric cofactor to carbamyl phosphate synthetase found in the cytoplasm, ... In vertebrae and mammals, N-acetylglutamic acid is the allosteric activator molecule to mitochondrial carbamyl phosphate ... carboxyl group of N-acetylglutamic acid using the phosphate produced by hydrolysis of adenosine triphosphate (ATP). Rhizobium ...

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

Carrey EA, Campbell DG, Hardie DG (1986). "Phosphorylation and activation of hamster carbamyl phosphate synthetase II by cAMP- ...

https://en.wikipedia.org/wiki/PPP1CA

Carrey EA, Campbell DG, Hardie DG (Dec 1985). "Phosphorylation and activation of hamster carbamyl phosphate synthetase II by ...

https://en.wikipedia.org/wiki/PPP2CA

Nyunoya H, Lusty CJ (August 1984). "Sequence of the small subunit of yeast carbamyl phosphate synthetase and identification of ... glutamine-dependent carbamoyl-phosphate synthase; phosphoribosylformylglycinamidine synthase II; and the histidine ...

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

"Regulation of aminotransferase-glutamate dehydrogenase interactions by carbamyl phosphate synthase-I, Mg2+ plus leucine versus ... Ford GC, Eichele G, Jansonius JN (May 1980). "Three-dimensional structure of a pyridoxal-phosphate-dependent enzyme, ... Glutamic-oxaloacetic transaminase is a pyridoxal phosphate-dependent enzyme which exists in cytoplasmic and inner-membrane ... "Three-dimensional structure of a pyridoxal-phosphate-dependent enzyme, mitochondrial aspartate aminotransferase". Proceedings ...

https://en.wikipedia.org/wiki/GOT2

The substrates of the reaction catalyzed by ornithine transcarbamylase are ornithine and carbamyl phosphate, while the product ... The increased orotic acid concentrations result from the buildup of carbamoyl phosphate. This biochemical phenotype (increased ... responsible for converting carbamoyl phosphate and ornithine into citrulline. OTC deficiency is inherited in an X-linked ...

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

Homology of putative targeting sequence to that of carbamyl phosphate synthetase I revealed by correlation of cDNA and protein ...

https://en.wikipedia.org/wiki/ALDH2

At present, it is thought that the depletion of the ornithine supply causes the accumulation of carbamyl-phosphate in the urea ...

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

His research was now focused on aspartate transcarbamylase, which catalyzes the transfer of a carbamyl group from phosphate to ...

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

... carbamyl phosphate MeSH D02.241.081.251.150 - carbaryl MeSH D02.241.081.251.165 - carisoprodol MeSH D02.241.081.251.240 - ... polyisoprenyl phosphates MeSH D02.455.849.690.250 - dolichol phosphates MeSH D02.455.849.690.700 - polyisoprenyl phosphate ... inositol phosphates MeSH D02.033.800.519.400.350 - inositol 1,4,5-trisphosphate MeSH D02.033.800.519.400.700 - phytic acid MeSH ... tritolyl phosphates MeSH D02.755.410.350 - gallic acid MeSH D02.755.410.350.299 - hydrolyzable tannins MeSH D02.755.410.350.600 ...

https://en.wikipedia.org/wiki/List_of_MeSH_codes_(D02)

... glucosamine-6-phosphate and carbamyl phosphate, alanine, and glycine. An excess of any one product does not individually ... After, His3 forms imidazole acetol-phosphate releasing water. His5 then makes L-histidinol-phosphate, which is then hydrolyzed ... The first step, condensation of 3-deoxy-D-arabino-heptulosonic acid 7-phosphate (DAHP) from PEP/E4P, uses three isoenzymes AroF ... Homoserine undergoes O-phosphorylation; this phosphate ester undergoes hydrolysis concomitant with relocation of the OH group. ...

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

... the condensation of l-aspartate and carbamoyl phosphate to form N-carbamyl-L-aspartate and inorganic phosphate. The catalysis ... and the carbamoyl phosphate domain, which contains most of the residues that bind to carbamoyl phosphate. Each regulatory ... These residues are located at the cleft between the carbamoyl phosphate and aspartate domains at the C1-C4 interface. The ... Gouaux JE, Lipscomb WN (Jun 1988). "Three-dimensional structure of carbamoyl phosphate and succinate bound to aspartate ...

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

... buchneri have been known to produce carbamyl phosphate and citrulline which can be precursors to ethyl carbamate formation. L. ... However, unlike wine yeast, lactic acid bacteria can not use the supplement diammonium phosphate as a nitrogen source. Before ... wine infected by Brettanomyces yeast in the presence of ammonium phosphate and lysine have also been known to exhibit this ...

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

Simmer JP, Kelly RE, Rinker AG, Scully JL, Evans DR (June 1990). "Mammalian carbamyl phosphate synthetase (CPS). DNA sequence ... Carbamoyl phosphate synthetase catalyzes the ATP-dependent synthesis of carbamoyl phosphate from glutamine (EC 6.3.5.5) or ... This enzyme catalyzes the reaction of ATP and bicarbonate to produce carboxy phosphate and ADP. Carboxy phosphate reacts with ... Carbamoyl phosphate synthetase III (found in fish). Carbamoyl phosphate synthase has three main steps in its mechanism and is, ...

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

Powers SG, Griffith OW, Meister A (May 1977). "Inhibition of carbamyl phosphate synthetase by P1, P5-di(adenosine 5')- ... Meister A (1989). "Mechanism and Regulation of the Glutamine-Dependent Carbamyl Phosphate Synthetase of Escherichia Coli". ... Mechanism and regulation of the glutamine-dependent carbamyl phosphate synthetase of Escherichia coli. Advances in Enzymology ... it is CPSI's job to add the ammonia to bicarbonate along with a phosphate group to form carbamoyl phosphate. Carbamoyl ...

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

Kalman SM, Duffield PH, Brzozowski T (April 1966). "Purification and properties of a bacterial carbamyl phosphate synthetase". ... Yip MC, Knox WE (May 1970). "Glutamine-dependent carbamyl phosphate synthetase. Properties and distribution in normal and ... 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 ...

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

Hong J, Salo WL, Lusty CJ, Anderson PM (October 1994). "Carbamyl phosphate synthetase III, an evolutionary intermediate in the ... Devaney MA, Powers-Lee SG (January 1984). "Immunological cross-reactivity between carbamyl phosphate synthetases I, II, and III ... Carbamoyl Phosphate synthetase III (CPS III) is one of the three isoforms of the Carbamoyl Phosphate Synthetase, en enzyme that ... Kong H, Kahatapitiya N, Kingsley K, Salo WL, Anderson PM, Wang YS, Walsh PJ (January 2000). "Induction of carbamoyl phosphate ...

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

... pyridoxal phosphate. Copper AJ, Meister A (1973). "Enzymatic conversion of O-carbamyl-L-serine to pyruvate and ammonia". ... Portal: Biology v t e (EC 4.3.1, Pyridoxal phosphate enzymes, Enzymes of unknown structure, All stub articles, Lyase stubs). ...

https://en.wikipedia.org/wiki/Carbamoyl-serine_ammonia-lyase

... glyceraldehyde 3-phosphate ⇌ {\displaystyle \rightleftharpoons } erythrose 4-phosphate + fructose 6-phosphate. Transfer of acyl ... Reichard P, Hanshoff G (1956). "Aspartate Carbamyl Transferase from Escherichia coli" (PDF). Acta Chemica Scandinavica. 10: 548 ... Groups that are classified as phosphate acceptors include: alcohols, carboxy groups, nitrogenous groups, and phosphate groups. ... Most symptoms of the disease are caused by a buildup of galactose-1-phosphate in the body. Common symptoms include liver ...

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

Grisolia, S; Cohen, P P (1952). "The catalytic role of carbamyl glutamate in citrulline biosynthesis". J. Biol. Chem. 198 (2): ... Rubio, V; Grisolia, S (1977). "Mechanism of mitochondrial carbamoyl-phosphate synthetase - synthesis and properties of active ... carbamoyl-phosphate synthetase and other enzymes. He was appointed Grand Cross of the Orden Civil de Sanidad (Civil Order of ... "Mechanism of activation of bicarbonate ion by mitochondrial carbamoyl-phosphate synthetase - formation of enzyme-bound ...

https://en.wikipedia.org/wiki/Santiago_Grisolía,_1st_Marquess_of_Grisolía

... carbamyl phosphate synthetase (CPS). Compound 2 is citrulline, the product of condensation between carbamyl phosphate (1) and ... Compounds that comprise the urea cycle are numbered sequentially, beginning with carbamyl phosphate. At the first step (1), the ...

https://emedicine.medscape.com/article/948084-treatment

Ammonul must be administered with arginine for carbamyl phosphate synthetase (CPS), ornithine transcarbamylase (OTC), ... Carglumic acid acts as an activator of carbamoyl phosphate synthetase (CPS 1), improves or restores the function of the urea ... disturbance in phosphate metabolism, Fanconi syndrome, bad taste, and offensive body odor. Available in powder and tablet forms ...

https://www.medscape.com/answers/1174503-159893/what-are-the-medication-recommendations-for-patients-with-hyperammonemia

... carbamoyl phosphate synthetase (CPS). Compound 2 is citrulline, which is the product of condensation between carbamyl phosphate ... Compounds that comprise the urea cycle are sequentially numbered, beginning with carbamyl phosphate (1). At this step, the ...

http://emedicine.medscape.com/article/950672-workup

... carbamyl phosphate synthetase (CPS). Compound 2 is citrulline, the product of condensation between carbamyl phosphate (1) and ... Compounds comprising the urea cycle are numbered sequentially, beginning with carbamyl phosphate (1). At this step, the first ... Diez-Fernandez C, Häberle J. Targeting CPS1 in the treatment of Carbamoyl phosphate synthetase 1 (CPS1) deficiency, a urea ...

https://emedicine.staging.medscape.com/article/941090-workup

Carbamoyl phosphate synthetase (CPS) deficiency is a urea cycle defect that results from a deficiency in an enzyme that ... carbamyl phosphate synthetase (CPS). Compound 2 is citrulline, the product of condensation between carbamyl phosphate (1) and ... Carbamyl-phosphate-synthetase deficiency with neonatal onset of symptoms. Acta Paediatr Scand. 1977 Jul. 66(4):529-34. [QxMD ... Carbamyl phosphate synthase deficiency: diagnosed during pregnancy in a 41-year-old. J Clin Neurosci. 2006. 13:702-6. ...

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

http://emedicine.medscape.com/article/950672-medication

... carbamyl phosphate synthetase (CPS). Compound 2 is citrulline, the product of condensation between carbamyl phosphate (1) and ... carbamyl phosphate synthetase (CPS). Compound 2 is citrulline, the product of condensation between carbamyl phosphate (1) and ... Compounds that comprise the urea cycle are numbered sequentially, beginning with carbamyl phosphate. At the first step (1), the ... Compounds that comprise the urea cycle are numbered sequentially, beginning with carbamyl phosphate. At the first step (1), the ...

http://emedicine.medscape.com/article/948084-overview

Carbamoyl phosphate synthetase I deficiency is an inherited disorder that causes ammonia to accumulate in the blood ( ... Gene structure of human carbamylphosphate synthetase 1 and novel mutations in patients with neonatal onset. Hum Mutat. 2003 Apr ... Mutations in the CPS1 gene cause carbamoyl phosphate synthetase I deficiency. The CPS1 gene provides instructions for making ... Carbamoyl phosphate synthetase I deficiency is a rare disorder; its overall incidence is unknown. Researchers in Japan have ...

https://medlineplus.gov/genetics/condition/carbamoyl-phosphate-synthetase-i-deficiency/

It begins with the formation of carbamyl phosphate from ammonia by carbamyl phosphate synthetase. Carbamyl phosphate is added ... CSPD, carbamyl phosphate synthetase deficiency; HHH, hyperornithinemia hyperammonemia homocitrullinuria syndrome; IVA, ... CSPD, carbamyl phosphate synthetase deficiency; HHH, hyperornithinemia hyperammonemia homocitrullinuria syndrome; IVA, ... carbamyl phosphate synthetase (CPS), ornithine transcarbamylase (OTC), argininosuccinic acidemia (AS), and argininosuccinic ...

https://www.medscape.com/viewarticle/465899_8

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

... a male neonate with hyperammonemia who showed progressive neurologic symptoms and pulmonary bleeding due to carbamyl phosphate ... is greatest in carbamoyl phosphate synthetase 1 deficiency (CPS1D, OMIM 608307) (42%), followed by OTCD (11%), argininosuccinic ...

https://www.researchgate.net/scientific-contributions/E-Ploechl-61920238

https://emedicine.medscape.com/article/950752-differential

... carbon dioxide and ammonia to yield carbamyl phosphate. Carbamyl phosphate reacts with ornithine to form citrulline which ... This cycle of reactions involves several enzymes including carbamyl phosphate synthetase, ornithine carbamylase, ...

https://inchem.org/documents/jecfa/jecmono/v32je16.htm

In addition, greater than 60% reduction in Vmax and Km for carbamyl phosphate was noted in all four patients in whom sample ... In addition, greater than 60% reduction in Vmax and Km for carbamyl phosphate was noted in all four patients in whom sample ... In addition, greater than 60% reduction in Vmax and Km for carbamyl phosphate was noted in all four patients in whom sample ... "ABNORMALITIES OF CARBAMYL-PHOSPHATE SYNTHETASE AND ORNITHINE TRANSCARBAMYLASE IN LIVER OF PATIENTS WITH REYES SYNDROME." ,i> ...

https://med.stanford.edu/profiles/Philip_Sunshine

Arginine deficiency leads to carbamyl phosphate overproduction in the mitochondria due to inadequate ornithine supply. Arginine ... Arginine deficiency syndrome is observed in human babies born with a phosphate synthetase deficiency. Normal growth and ...

https://nutritionreview.org/amino-acids/

... carbamyl phosphate; GLN, glutamine; GMP; guanosine 5-monophosphate, IMP; inosine 5-monophosphate; ORN, ornithine; PPi, ... A block distal to the formation of mitochondrial carbamoyl phosphate (CP) in the urea cycle results in excessive excretion of ...

https://jofem.org/index.php/jofem/article/view/209/265

Import of carbamylphosphate synthetase and ornithine transcarbamylase into mitochondria of rat liver: detection of aggre... ... Electrophoretic mobility patterns of four enzymes, viz., EC 5.3.1.9 glucose phosphate isomerase (GPI); EC 1.1.1,4.0.L-malate; ... We investigated the distribution of the nuclear encoded mitochondrial enzymes, carbamylphosphate synthetase (CPS; EC 6.3.4.16) ...

https://www.mysciencework.com/search/publications?facets%5Bjournal%5D%5B0%5D=The%20Australian%20journal%20of%20experimental%20biology%20and%20medical%20science&page=5

... carbamyl phosphate synthetase deficiency (237300), argininosuccinate synthetase deficiency, or citrullinemia (215700), ...

http://mips.helmholtz-muenchen.de/phenodis/disease/show/2495

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

Carbamyl phosphate synthetase deficiency (CPS). • Ornithine transcarbamylase deficiency (OTC). • Argininosuccinic acid ...

https://www.brainkart.com/article/Paediatrics--Urea-cycle-disorders_29110/

Carbamyl Phosphate 100% * pyrimidine 79% * Lung Neoplasms 50% * phosphorylethanolamine 47% * DNA 35% ...

https://utsouthwestern.pure.elsevier.com/en/organisations/childrens-medical-center-research-institute-at-ut-southwestern/publications/?type=%2Fdk%2Fatira%2Fpure%2Fresearchoutput%2Fresearchoutputtypes%2Fcontributiontojournal%2Fcomment

Characterization and derivation of the gene coding for mitochondrial carbamyl phosphate synthetase 1 of rat. Nyunoya, H., ...

https://waseda.pure.elsevier.com/ja/publications/?ordering=title&descending=true&showAdvanced=false&allConcepts=true&inferConcepts=true&originalSearch=&improvedLayoutOrganisationUuid=&format=&page=1257

... carbamyl phosphate synthetase (CPS). Compound 2 is citrulline, the product of condensation between carbamyl phosphate (1) and ... carbamyl phosphate synthetase (CPS). Compound 2 is citrulline, the product of condensation between carbamyl phosphate (1) and ... The normal function of N-acetylglutamate (NAG), the reaction product, is to act as an activator of carbamyl phosphate ... Compounds comprising the urea cycle are numbered sequentially, beginning with carbamyl phosphate (1). At this step, the first ...

https://reference.staging.medscape.com/article/941090-overview

Urea Cycle Disorders, Inborn;Inborn Errors of Metabolism;Propionic Acidemia;Methylmalonic Acidemia;Carbamyl Phosphate ... Propionic Acidemia, Type I and/or Type II;Methylmalonic Acidemia;Carbamoyl-Phosphate Synthase I Deficiency Disease;Ornithine ...

https://ddrare.nibiohn.go.jp/ddrare_Mar2021/cgi-bin/disease_who.cgi?id=246

Carbamyl Phosphate 100% * pyrimidine 79% * Lung Neoplasms 50% * phosphorylethanolamine 47% * DNA 35% ...

https://utsouthwestern.pure.elsevier.com/en/organisations/pediatric-residency-2/publications/?type=%2Fdk%2Fatira%2Fpure%2Fresearchoutput%2Fresearchoutputtypes%2Fcontributiontojournal%2Fcomment

Small subunit of carbamyl phosphate synthetase is related to component II of anthranilate and p-aminobenzoic acid synthetases. ... Sequence of the small subunit of yeast carbamyl phosphate synthetase and identification of its catalytic domain. Nyunoya, H. & ... Photomicrobial sensors for selective determination of phosphate. Matsunaga, T., Suzuki, T. & Tomoda, R., 1984, In: Enzyme and ...

https://waseda.pure.elsevier.com/en/publications/?format=&page=1469&ordering=publicationYearThenTitle&descending=true

Relation of Macrophage Migration Inhibitory Factor to Pulmonary Hemodynamics and Vascular Structure and Carbamyl-Phosphate ... Association between genetic variations in carbamoyl-phosphate synthetase gene and persistent neonatal pulmonary hypertension. ...

https://phgkb.cdc.gov/PHGKB/searchSummary.action?firstQuery=Pulmonary+Hypertension&Mysubmit=Search&dbTypeChoice=HLBS

To assay Carbamyl Phosphate Synthetase, citrulline formation from ammonia, bicarbonate and Ornithine is measured, to assay ... By assaying Carbamyl Phosphate Synthetase (CPS) and Ornithine Transcarbamylase (OTC), citrulline produc... ... This pathway utilises the enzymes Carbamyl Phosphate Synthetase, Ornithine Transcarbamylase, Arginine Synthetase and Arginase ... citrulline formation from Ornithine and Carbamyl phosphate is measured.. Arginine Synthetase is assayed by measuring urea ...

http://termpaperaccess.com/index.php?act=doc_enzymes_of_the_urea_cycle_frudw

... carbamyl phosphate synthetase 1 (CPS1). However, despite the important role of NAGS in ammonia removal, little is known about ... carbamyl phosphate synthetase 1 (CPS1). However, despite the important role of NAGS in ammonia removal, little is known about ... Amino-Acid-N-Acetyltransferase--genetics; Carbamoyl-Phosphate Synthase (Ammonia)--biosynthesis; Carbamoyl-Phosphate Synthase ( ...

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

YJR109C 1533.292175 INESSENTIAL CPA2 carbamyl phosphate synthetase, arginine biosynthesis, carbamoyl-phosphate synthase ( ... 3.429802 INESSENTIAL PHO84 inorganic phosphate transporter, transmembrane protein, phosphate transport, inorganic phosphate ... alpha-trehalose-phosphate synthase (UDP-forming), alpha,alpha-trehalose-phosphate synthase (UDP-forming) YHR198C -3.278204 ... phosphate oxidase,pyridoxamine-phosphate oxidase, YPR011C 38.558605 INESSENTIAL biological_process unknown, molecular_function ...

https://genomics.lbl.gov/YeastFitnessData/websitefiles/47_minimal-media_sen.txt

Carbamyl Phosphate. *Chlorfenvinphos. *Dichlorvos. *Diphosphoglyceric Acids. *Diphosphonates. *Fosinopril. *Hempa. * ...

https://connects.catalyst.harvard.edu/Profiles/display/Concept/Diphosphonates

Stimulation of the ATP synthesis reaction of biotin carboxylase where carbamyl phosphate reacted with ADP by holoBCCP87 was 5- ... Carbamoyl phosphate synthetase (CPS) catalyzes the formation of carbamoyl phosphate from glutamine, bicarbonate, and 2 mol of ... Role of conserved residues within the carboxy phosphate domain of carbamoyl phosphate synthetase. ... The crystals are grown by microdialysis against 10 mM potassium phosphate (pH 7.0), 1 mM EDTA, 2 mM DTT and 1 mM NaN3 at 4 ...

https://smart.embl.de/smart/show_secondary.cgi?domain=Biotin_carb_C

Examples are ornithine transcarbamylase deficiency, carbamyl phosphate synthetase deficiency, and citrullinemia. Ornithine ... Galactosemia results from the deficiency of galactose-1-phosphate uridylyltransferase, which leads to an accumulation of ... Acetyl CoA = acetyl coenzyme A; AL = argininosuccinate lyase; ASS = argininosuccinate synthase; CPS = carbamoyl phosphate ... galactose-1-phosphate and other metabolites that are toxic to the liver. In addition to hepatic dysfunction, these infants may ...

https://accessemergencymedicine.mhmedical.com/content.aspx?bookid=1658§ionid=109410906

Carbamyl phosphate (CP) produced by yeast (Saccharomyces cerevisiae) can react with ethanol www.selleckchem.com/products/GDC- ... Intermediates such as carbamyl phosphate (CH4NO5P) are also easily formed in vitro. The results for EC content in the each ... Membranes were blocked with 2.5% (w/v) nonfat dry milk in phosphate buffered saline (PBS) with 0.1% Tween 20 and probed ... CP comes from arginine, catalysed by carbamyl synthase, involving ATP, CO2, and ammonia ( Ingledew, Magnus, & Patterson, 1987 ...

https://sirtsignaling.com/2017/08

... carbamoyl phosphate synthetase (CPS). Compound 2 is citrulline, which is the product of condensation between carbamyl phosphate ... carbamoyl phosphate synthetase (CPS). Compound 2 is citrulline, which is the product of condensation between carbamyl phosphate ... carbamoyl phosphate synthetase (CPS). Compound 2 is citrulline, which is the product of condensation between carbamyl phosphate ... Failure to incorporate carbamyl phosphate into citrulline by condensation with ornithine results in an excess of both ...

https://emedicine.staging.medscape.com/article/950672-overview

Carbamoyl phosphate synthetase I deficiency is an inherited disorder that causes ammonia to accumulate in the blood (hyperammonemia). (medlineplus.gov)
In the first few days of life, infants with carbamoyl phosphate synthetase I deficiency typically exhibit the effects of hyperammonemia, which may include unusual sleepiness, poorly regulated breathing rate or body temperature, unwillingness to feed, vomiting after feeding, unusual body movements, seizures, or coma. (medlineplus.gov)
In some people with carbamoyl phosphate synthetase I deficiency, signs and symptoms may be less severe and appear later in life. (medlineplus.gov)
Mutations in the CPS1 gene cause carbamoyl phosphate synthetase I deficiency. (medlineplus.gov)
Carbamoyl phosphate synthetase I deficiency belongs to a class of genetic diseases called urea cycle disorders. (medlineplus.gov)
Ammonia is especially damaging to the brain, and excess ammonia causes neurological problems and other signs and symptoms of carbamoyl phosphate synthetase I deficiency. (medlineplus.gov)
Finckh U, Kohlschutter A, Schafer H, Sperhake K, Colombo JP, Gal A. Prenatal diagnosis of carbamoyl phosphate synthetase I deficiency by identification of a missense mutation in CPS1. (medlineplus.gov)
Arginine deficiency syndrome is observed in human babies born with a phosphate synthetase deficiency. (nutritionreview.org)
Arginine deficiency leads to carbamyl phosphate overproduction in the mitochondria due to inadequate ornithine supply. (nutritionreview.org)
A trained nutritionist should scrupulously monitor the diet of a patient with carbamoyl phosphate synthetase (CPS) deficiency. (medscape.com)
Understanding carbomyl phosphate synthetase deficiency: impact of clinical mutations on enzyme functionality. (medscape.com)
Understanding carbomyl phosphate synthetase (CPS1) deficiency by using the recombinantly purified human enzyme: effects of CPS1 mutations that concentrate in a central domain of unknown function. (medscape.com)
Carbamoyl phosphate synthetase 1 deficiency in Italy: clinical and genetic findings in a heterogeneous cohort. (medscape.com)
Postpartum coma and death due to carbamoyl-phosphate synthetase I deficiency. (medscape.com)
this results in failure to activate the enzyme responsible for the reaction NH 4 + + CO 2 + ATP → H 2 N-CO-PO 3 2- + ADP, which is the entry step into the urea cycle (see Carbamyl Phosphate Synthetase Deficiency ). (medscape.com)
Clinical signs and symptoms of NAGS deficiency occur when ammonia fails to fix into carbamoyl phosphate (CP) effectively, thus disabling the urea cycle. (medscape.com)

Carbamyl phosphate reacts with ornithine to form citrulline which combines with aspartate to form argininosuccinate. (inchem.org)
citrulline formation from Ornithine and Carbamyl phosphate is measured. (termpaperaccess.com)
By assaying Carbamyl Phosphate Synthetase (CPS) and Ornithine Transcarbamylase (OTC), citrulline produc. (termpaperaccess.com)
A mutant enzyme protein impairs the reaction that leads to condensation of carbamyl phosphate and ornithine to form citrulline. (medscape.com)
PHENOTYPE: In the absence of N-carbamyl-L-glutamate and L-citrulline supplementation homozygous null mice develop severe hyperammonemia and die. (utsouthwestern.edu)

The specific role of the carbamoyl phosphate synthetase I enzyme 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. (medlineplus.gov)
In this condition, the carbamoyl phosphate synthetase I enzyme is at low levels (deficient) or absent, and the urea cycle cannot proceed normally. (medlineplus.gov)
Carbamoyl phosphate synthetase (glutamine-hydrolysing) (EC 6.3.5.5) is an enzyme that catalyzes the reactions that produce carbamoyl phosphate in the cytosol (as opposed to type I, which functions in the mitochondria). (wikipedia.org)
Carbamoyl phosphate synthetase I Carbamoyl phosphate synthetase III Anderson PM, Meister A (December 1965). (wikipedia.org)
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)
Association between genetic variations in carbamoyl-phosphate synthetase gene and persistent neonatal pulmonary hypertension. (cdc.gov)
The disorder is caused by a reduction in the activity of hepatic mitochondrial CARBAMOYL-PHOSPHATE SYNTHASE (AMMONIA ). (nih.gov)

Compounds that comprise the urea cycle are numbered sequentially, beginning with carbamyl phosphate. (medscape.com)
The N-acetylglutamate synthase gene encodes a mitochondrial enzyme that catalyzes the formation of N-acetylglutamate (NAG) from glutamate and acetyl coenzyme-A. NAG is a cofactor of carbamyl phosphate synthetase I (CPSI), the first enzyme of the urea cycle in mammals. (utsouthwestern.edu)

The normal function of N -acetylglutamate (NAG), the reaction product, is to act as an activator of carbamyl phosphate synthetase (CPS), which is also a mitochondrial enzyme. (medscape.com)
A signal sequence domain essential for processing, but not import, of mitochondrial pre-ornithine carbamyl transferase. (mcmaster.ca)

also at this step, N-acetylglutamate exerts its regulatory control on the mediating enzyme, carbamyl phosphate synthetase (CPS). (medscape.com)
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)

This is a cyclic process in which the initial step is the reaction between carbon dioxide and ammonia to yield carbamyl phosphate. (inchem.org)

This cycle of reactions involves several enzymes including carbamyl phosphate synthetase, ornithine carbamylase, argininosuccinate synthetase and arginine-lyase. (inchem.org)
This pathway utilises the enzymes Carbamyl Phosphate Synthetase, Ornithine Transcarbamylase, Arginine Synthetase and Arginase in varying amounts to catalyse reactions. (termpaperaccess.com)

Import of carbamylphosphate synthetase and ornithine transcarbamylase into mitochondria of rat liver: detection of aggre. (mysciencework.com)

The model was then used to simulate a set of perturbed conditions as follows: genetic derepression, feedback resistance of the first enzymatic step, and low constitutive synthesis of the intermediate carbamyl phosphate. (unipr.it)

Molecular defects in human carbamoy phosphate synthetase I: mutational spectrum, diagnostic and protein structure considerations. (medscape.com)

Carbamyl phosphate synthetase. (wikidata.org)
7)Valproic acid given to patients with urea cycle disorders may exacerbate their condition and antagonize the efficacy of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% through inhibition of the synthesis of N-acetylglutamate, co-factor for carbamyl phosphate synthetase. (drugcentral.org)
also at this step, N-acetylglutamate exerts its regulatory control on the mediating enzyme, carbamyl phosphate synthetase (CPS). (medscape.com)
Sequence of the small subunit of yeast carbamyl phosphate synthetase and identification of its catalytic domain. (expasy.org)
Carbamoyl phosphate synthetase I is a ligase enzyme located in the mitochondria involved in the production of urea. (csnpharm.cn)
Carbamoyl phosphate synthetase I (CPS1 or CPSI) transfers an ammonia molecule from glutamine or glutamate to a molecule of bicarbonate that has been phosphorylated by a molecule of ATP. (csnpharm.cn)
H3B-120 is a highly selective, competitive and allosteric carbamoyl phosphate synthetase 1 (CPS1) inhibitor with an IC50 of 1.5 μM and a Ki of 1.4 μM. (csnpharm.cn)
Carglumic acid (N-Carbamyl-L-glutamic acid), a functional analogue of N-acetylglutamate (NAG) and a carbamoyl phosphate synthetase 1 (CPS1) activator, is used to treat acute and chronic hyperammonemia associated with NAG synthase (NAGS) deficiency. (csnpharm.cn)
Heliconiinae carbamyl P-synthetase (Cad) gene, partial cds. (beds.ac.uk)

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

Heliconiinae glyceraldehyde 3-phosphate dehydrogenase (Gapdh) gene, partial cds. (beds.ac.uk)

Cloning and sequence of a cDNA encoding human carbamyl phosphate synthetase I: molecular analysis of hyperammonemia. (nih.gov)
Carbamoyl phosphate synthetase I deficiency is an inherited disorder that causes ammonia to accumulate in the blood (hyperammonemia). (nih.gov)
In the first few days of life, infants with carbamoyl phosphate synthetase I deficiency typically exhibit the effects of hyperammonemia, which may include unusual sleepiness, poorly regulated breathing rate or body temperature, unwillingness to feed, vomiting after feeding, unusual body movements, seizures, or coma. (nih.gov)
Carbamoyl phosphate synthetase I deficiency (CPS1 deficiency) is a genetic disorder that causes episodes of toxic levels of ammonia in the blood (hyperammonemia). (rareginews.com)
The overall objective of this drug trial is to determine whether the treatment of acute hyperammonemia with N-carbamyl-L-glutamate (NCG, Carglumic acid) in propionic acidemia (PA), methylmalonic acidemia (MMA), late-onset CPS1 deficiency (CPSD) and late-onset Ornithine transcarbamylase deficiency (OTCD) accelerates the resolution of hyperammonemia efficiently and safely. (clinicaltrials.gov)

Furthermore, the sequence displays a homology, when centered around the cleavage site, with the leader sequence of rat liver carbamyl phosphate synthetase I, suggesting a novel similarity between mitochondrial targeting sequences of two different enzymes. (drugbank.com)
Kinetic studies on rat liver carbamyl phosphate synthetase. (houstonmethodist.org)

Carbamoyl-phosphate synthase catalyses the ATP-dependent synthesis of carbamyl-phosphate from glutamine or ammonia and bicarbonate. (embl-heidelberg.de)
The carbamoyl-phosphate synthase (CPS) enzyme in prokaryotes is a heterodimer of a small and large chain. (embl-heidelberg.de)

Valproic acid given to patients with urea cycle disorders may exacerbate their condition and antagonize the efficacy of AMMONUL through inhibition of the synthesis of N- acetylglutamate, a co-factor for carbamyl phosphate synthetase. (nih.gov)
In vivo synthesis of carbamyl phosphate from NH3 by the large subunit of Escherichia coli carbamyl phosphate synthetase. (elsevier.com)
The elevated levels of the large subunit enable the transformed cells to utilize NH3 but not glutamine as nitrogen donor for carbamyl phosphate synthesis. (elsevier.com)

A mutant E. coli strain lacking both subunits of carbamyl phosphate synthetase when transformed with pHN12 overproduces the large subunit by 200-fold (8-10% of the cellular protein). (elsevier.com)
This entry represents the oligomerisation domain found in the large subunit of carbamoyl phosphate synthases as well as in certain other carboxy phosphate domain-containing enzymes. (embl-heidelberg.de)
The large subunit consists of fourstructural units: the carboxyphosphate synthetic component, theoligomerization domain, the carbamoyl phosphate synthetic component andthe allosteric domain. (embl-heidelberg.de)

Aspartate transcarbamylase activity was assayed in rat tissues, and the limitation caused in some tissues by carbamyl phosphate hydrolysis was avoided. (aacrjournals.org)

J. 33, 1942 (1939) Carglumic acid is a Carbamoyl Phosphate Synthetase 1 Activator. (drugapprovalsint.com)

The rate-limiting step is carbamoyl phosphate synthetase (CPS) disposal of waste nitrogen. (medscape.com)
Previous results have indicated that under conditions of nitrogen excess, transcriptional activation is prevented by an NRI-phosphate phosphatase activity that is observed when NRII and another signal transduction protein known as PII (the glnB product) interact. (versila.com)

Homology of putative targeting sequence to that of carbamyl phosphate synthetase I revealed by correlation of cDNA and protein data. (drugbank.com)

The main synthetic groups are the chlorinated hydrocarbons, organic phosphates organophosphates , and carbamates. (alpost103.org)

Alr1p contains pyridoxal 5'-phosphate as a coenzyme and catalyzes the racemization of alanine with apparent K(m) and V(max) values as follows: for L-alanine, 5.0 mM and 670 micromol/min/mg, respectively, and for D-alanine, 2.4 mM and 350 micromol/min/mg, respectively. (omicsdi.org)
During the reaction of cystalysin with L- or D-alanine, a time-dependent loss of beta-elimination activity occurs concomitantly with the conversion of the pyridoxal 5'-phosphate (PLP) coenzyme into pyridoxamine 5'-phosphate (PMP). (omicsdi.org)

Purification and kinetic properties of brain orotidine 5'-phosphate decarboxylase. (houstonmethodist.org)

Regulation of carbamoyl phosphate synthetase by MAP kinase. (cocites.com)

The cloned carAB operon of Escherichia coli coding for the small and large subunits of carbamyl phosphate synthetase has been used to construct a recombinant plasmid with a 4.16 kilobase ClaI fragment of the car operon that lacks the major promoters, P1 and P2. (elsevier.com)

In the carbamoyl phosphate synthetic component,the single observed Mn2+ ion is also octahedrally coordinated byoxygen-containing ligands and Glu761 plays a similar role to that ofGlu215. (embl-heidelberg.de)
The carboxyphosphate and carbamoyl phosphate synthetic components,while topologically equivalent, are structurally different, as would beexpected in light of their separate biochemical functions. (embl-heidelberg.de)

Pyruvate replaced acetate as the major excreted product, and the following intermediates of glycolysis were excreted in significant amounts: glucose-6-phosphate, fructose-1 ,6-diphos- phate, dihydroxyacetone phosphate, and 3-phosphoglycerate. (nih.gov)

The human Rad9 checkpoint protein stimulates the carbamoyl phosphate synthetase activity of the multifunctional protein CAD. (cocites.com)

Phosphorylation and activation of hamster carbamyl phosphate synthetase II by cAMP-dependent protein kinase. (cocites.com)

The structure of carbamoyl phosphate synthetase determined to 2.1 Aresolution. (embl-heidelberg.de)

This graph shows the total number of publications written about "Polyisoprenyl Phosphates" by people in this website by year, and whether "Polyisoprenyl Phosphates" was a major or minor topic of these publications. (jefferson.edu)
Below are the most recent publications written about "Polyisoprenyl Phosphates" by people in Profiles. (jefferson.edu)

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Ca-releasing action of beta, gamma-methylene adenosine triphosphate on fragmented sarcoplasmic reticulum. (1/122)

Channeling of carbamoyl phosphate to the pyrimidine and arginine biosynthetic pathways in the deep sea hyperthermophilic archaeon Pyrococcus abyssi. (2/122)

Carbamate kinase: New structural machinery for making carbamoyl phosphate, the common precursor of pyrimidines and arginine. (3/122)

The carbamoyl-phosphate synthetase of Pyrococcus furiosus is enzymologically and structurally a carbamate kinase. (4/122)

Half of Saccharomyces cerevisiae carbamoyl phosphate synthetase produces and channels carbamoyl phosphate to the fused aspartate transcarbamoylase domain. (5/122)

Studies of hepatic glutamine metabolism in the perfused rat liver with (15)N-labeled glutamine. (6/122)

Substitutions in the aspartate transcarbamoylase domain of hamster CAD disrupt oligomeric structure. (7/122)

Antitumor activity of N-(phosphonacetyl)-L-aspartic acid, a transition-state inhibitor of aspartate transcarbamylase. (8/122)

Carbamyl Phosphate

Carbamoyl-Phosphate Synthase (Ammonia)

Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing)

Carbamates

Phosphates

Dihydroorotase

Aspartate Carbamoyltransferase

Ornithine Carbamoyltransferase

Ligases

Phosphotransferases

Ornithine

Ammonia

Argininosuccinate Synthase

Amino Acid Metabolism, Inborn Errors

Mitochondria, Liver

Glutamine

Kinetics

Glyoxal

Glutaminase

Uracil Nucleotides

Phosphoribosyl Pyrophosphate

Uridine Monophosphate

Carboxyl and Carbamoyl Transferases

Citrulline

Acetylene

Organophosphates

Escherichia coli

Adenosine Triphosphate

Amino-Acid N-Acetyltransferase

Organophosphorus Compounds

Calcium Phosphates

Glutamates

Phosphonoacetic Acid

Bicarbonates

Urea

Glucose-6-Phosphate

Glyceraldehyde-3-Phosphate Dehydrogenases

Arginine

Sugar Phosphates

Uracil

Liver

Oligomycins

Multienzyme Complexes

Dinitrophenols

Enzyme Repression

Mesocricetus

Molecular Weight

Hydrogen-Ion Concentration

Magnesium

Binding Sites

Clostridium

Inositol Phosphates

Cell-Free System

Orotic Acid

Cricetinae

Amino Acids

Amino Acid Sequence

Hydrolysis

Adenosine Diphosphate

Rats, Inbred Strains

Cloning, Molecular

Structure-Activity Relationship

Glyceraldehyde 3-Phosphate

Base Sequence

Pentose Phosphate Pathway

Molecular Sequence Data

Mutation

Glucosephosphate Dehydrogenase

Macromolecular Substances

DNA

Dihydroxyacetone Phosphate

Phosphate Transport Proteins

Pyridoxal Phosphate

Glucose-6-Phosphate Isomerase

Protein Binding

Genes

Glucosephosphates

Glycerol-3-Phosphate O-Acyltransferase

Sphingosine

Pentosephosphates