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).
Lists of words, usually in alphabetical order, giving information about form, pronunciation, etymology, grammar, and meaning.
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.
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.
Works containing information articles on subjects in every field of knowledge, usually arranged in alphabetical order, or a similar work limited to a special field or subject. (From The ALA Glossary of Library and Information Science, 1983)
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 nitrogenous groups, primarily amino groups, from a donor, generally an amino acid, to an acceptor, usually a 2-oxoacid. EC 2.6.
The smallest continent and an independent country, comprising six states and two territories. Its capital is Canberra.
Errors in metabolic processes resulting from inborn genetic mutations that are inherited or acquired in utero.
Health services, public or private, in rural areas. The services include the promotion of health and the delivery of health care.
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.
Studies designed to assess the efficacy of programs. They may include the evaluation of cost-effectiveness, the extent to which objectives are met, or impact.
The identification of selected parameters in newborn infants by various tests, examinations, or other procedures. Screening may be performed by clinical or laboratory measures. A screening test is designed to sort out healthy neonates (INFANT, NEWBORN) from those not well, but the screening test is not intended as a diagnostic device, rather instead as epidemiologic.
Errors in the metabolism of LIPIDS resulting from inborn genetic MUTATIONS that are heritable.
A fulminating bacterial infection of the deep layers of the skin and FASCIA. It can be caused by many different organisms, with STREPTOCOCCUS PYOGENES being the most common.
Anti-inflammatory agents that are non-steroidal in nature. In addition to anti-inflammatory actions, they have analgesic, antipyretic, and platelet-inhibitory actions.They act by blocking the synthesis of prostaglandins by inhibiting cyclooxygenase, which converts arachidonic acid to cyclic endoperoxides, precursors of prostaglandins. Inhibition of prostaglandin synthesis accounts for their analgesic, antipyretic, and platelet-inhibitory actions; other mechanisms may contribute to their anti-inflammatory effects.
Substances that reduce or suppress INFLAMMATION.
A genus of gram-positive, coccoid bacteria whose organisms occur in pairs or chains. No endospores are produced. Many species exist as commensals or parasites on man or animals with some being highly pathogenic. A few species are saprophytes and occur in the natural environment.
A non-steroidal anti-inflammatory agent (NSAID) with antipyretic and analgesic actions. It is primarily available as the sodium salt.
A nonsteroidal anti-inflammatory agent with analgesic properties used in the therapy of rheumatism and arthritis.
An anti-inflammatory agent with analgesic and antipyretic properties. Both the acid and its sodium salt are used in the treatment of rheumatoid arthritis and other rheumatic or musculoskeletal disorders, dysmenorrhea, and acute gout.
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.
The enzyme catalyzing the formation of orotidine-5'-phosphoric acid (orotidylic acid) from orotic acid and 5-phosphoribosyl-1-pyrophosphate in the course of pyrimidine nucleotide biosynthesis. EC 2.4.2.10.
Orotidine-5'-phosphate carboxy-lyase. Catalyzes the decarboxylation of orotidylic acid to yield uridylic acid in the final step of the pyrimidine nucleotide biosynthesis pathway. EC 4.1.1.23.
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.
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.
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 inherited urea cycle disorder associated with deficiency of the enzyme ORNITHINE CARBAMOYLTRANSFERASE, transmitted as an X-linked trait and featuring elevations of amino acids and ammonia in the serum. Clinical features, which are more prominent in males, include seizures, behavioral alterations, episodic vomiting, lethargy, and coma. (Menkes, Textbook of Child Neurology, 5th ed, pp49-50)

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)

Define carbamyl. carbamyl synonyms, carbamyl pronunciation, carbamyl translation, English dictionary definition of carbamyl. n a radical, NH2CO, that is derived from carbamic acid
Rate limiting enzymes Glycolysis- Phosphofructokinase 1f Gluconeogenesis- Fructose 1,6 bisphosphatase Glycogen synthesis- Glycogen synthase Glycogenolysis-Glycogen Phosphorylase Fatty acid synthesis- AcetylCoA Carboxylase Fatty acid beta oxidation-Carnitine acyl transferase 1 Lipolysis- hormone sensitive lipase Purine metabolism- PRPP Amidotransferase Pyrimidine metabolism- Aspartate transacetylase Ketone body synthesis-HMG CoA synthase Cholesterol synthesis- HMG CoA resuctase Bile acid synthesis- 7 Alpha hydroxylase Uric acid synthesis- xanthine oxidase Catecholamine synthesis- Tyrosine hydroxylase Urea cycle- Carbamoyl Phosphate synthase 1 Pentose phosphate pathway- Glucose-6-Phosphate dehydrogenase Krebs- Isocitrate dehydrogenase Adrenal hormones- Desmolase Porphyrin/Haem synthesis- ALA synthase Postaglandin synthesis- PG ...
Fingerprint Dive into the research topics of Synthesis of 5,10,15-Tris(αpha;, αpha;, αpha;-o-pivalamidophenyl)-20-[αpha;-o-[4-[[12- (1-imidazolyl) dodecyl]carbamoyl]-2,2-dimethyIbutanamido] phenyl]porphyrinatoiron (II). Together they form a unique fingerprint. ...
N-(N-((S)-1,3-Dicarboxypropyl)carbamoyl)-4-(18F)fluorobenzyl-L-cysteine: an imaging probe for prostate cancer; structure in first source
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).[2][3][6] CPSase has three active sites, one in the small subunit and two in the large subunit. The small subunit contains the glutamine binding site and catalyses the hydrolysis of glutamine to glutamate and ammonia, which is in turn used by the large chain to synthesize carbamoyl phosphate. The small subunit has a 3-layer beta/beta/alpha structure, and is thought to be mobile in most proteins that carry it. The C-terminal domain of the small subunit of CPSase has glutamine amidotransferase activity. The large subunit has two homologous carboxy phosphate domains, both of which have ATP-binding sites; however, the N-terminal carboxy phosphate domain catalyses the phosphorylation of biocarbonate, while the C-terminal domain catalyses the ...
1DUV: Mechanism of inactivation of ornithine transcarbamoylase by Ndelta -(N-Sulfodiaminophosphinyl)-L-ornithine, a true transition state analogue? Crystal structure and implications for catalytic mechanism.
1DUV: Mechanism of inactivation of ornithine transcarbamoylase by Ndelta -(N-Sulfodiaminophosphinyl)-L-ornithine, a true transition state analogue? Crystal structure and implications for catalytic mechanism.
A study of the sulfhydryl groups of the catalytic subunit of Escherichia coli aspartate transcarbamylase. The use of enzyme--5-thio-2-nitrobenzoate mixed disulfides as intermediates in modifying enzyme sulfhydryl groups ...
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Orotic acid, first discovered in ruminant milk, is an intermediate in the pyrimidine biosynthesis pathway of animal cells. Its synthesis is initiated by the formation of carbamoyl phosphate (CP) in the cytoplasm, with ammonia derived from glutamine.
Structure, properties, spectra, suppliers and links for: N-(3-Chloro-4-fluorophenyl)-2-{[2-({[3-(trifluoromethyl)phenyl]carbamoyl}amino)-3-pyridinyl]oxy}.
You are viewing an interactive 3D depiction of the molecule n-[(2-chloroethyl)(nitroso)carbamoyl]-l-alanyl-l-alanine (C9H15ClN4O5) from the PQR.
A realistic estimate of the Na+ entry needed is obtained by quadrupling this to take account of simultaneous activation of Na+ and K+ channels (Hodgkin, 1975), resulting in 11.5×10^8Na+ which have to be pumped out again, requiring 3.84×10^8ATP molecules to be hydrolyzed (Figs. 1B, 2, and 3 ...
CAMPAIGNERS from Wales took part in the Mass Action at Hinkley Point in Somerset calling for a halt to nuclear energy expansion.. CND Cymru vice-chairman Ray Davies, from Bedwas, and Paul Ralph, from Cwmbran, who campaign non-violently to rid the world of nuclear weapons, said they entered the proposed construction site at Hinkley nuclear power station in Somerset at 4am yesterday.. They managed to get through the fences to spread wildflower seeds.. Altogether about 30 protesters entered the site, displaying banners, and six people have been arrested. ...
PRINCIPLE: Enzymatic Assay of ORNITHINE CARBAMYL TRANSFERASE Carbamyl phosphate + L-Ornithine OCT > L-Citrulline + P i Abbreviations: P i = Inorganic Phosphate OCT = Ornithine Carbamyl Transferase CONDITIONS:
carbamyl plasmin A: an active enzyme with a single free NH(2)-terminal amino group(Val-561); derivative of EC 3.4.21.7, fibrinolysin
You are viewing an interactive 3D depiction of the molecule n-{2-benzyl-4-[(methylsulfonyl)carbamoyl]phenyl}-6-(cyclohexylmethyl)-2-pyridinecarboxamide (C28H31N3O4S) from the PQR.
A method for fabricating an LCD includes the steps of (a) loading a first substrate and a second substrate having seals formed thereon on a bonding chamber, (b) bonding the first and second substrates, (c) fixing the bonded first and second substrates, and (d) unloading the fixed first and second substrates.
Rat liver ornithine carbamoyltransferase appears to be located exclusively in the mitochondria; the activity that is found in the soluble fraction is indistinguishable from mitochondrial ornithine carbamoyltransferase by simple kinetic criteria, and seems to result from breakage of mitochondria during homogenization. Of several rat tissues studied, only the liver and the mucosa of small intestine contain significant amounts of ornithine carbamoyltransferase; the activity in intestinal mucosa is less than one thousandth of that in liver. Qualitatively, this distribution coincides with that of carbamoyl phosphate synthetase I and its cofactor, acetylglutamate. The rat liver contents of carbamoyl phosphate and ornithine were 0.1 and 0.15μmol/g wet wt. of tissue respectively. On the basis of these values, it is proposed that in vivo the ornithine carbamoyltransferase activity of liver may be much lower than its maximal activity in vitro might suggest.. ...
New treatments need to be developed for the significant human diseases of toxoplasmosis and malaria to circumvent problems with current treatments and drug resistance. Apicomplexan parasites causing these lethal diseases are deficient in pyrimidine salvage, suggesting that selective inhibition of de novo pyrimidine biosynthesis can lead to a severe loss of uridine 5-monophosphate (UMP) and thymidine 5-monophosphate (dTMP) pools, thereby inhibiting parasite RNA and DNA synthesis. Disruption of Toxoplasma gondii carbamoyl phosphate synthetase II (CPSII) induces a severe uracil auxotrophy with no detectable parasite replication in vitro and complete attenuation of virulence in mice. Here we show that a CPSII cDNA minigene efficiently complements the uracil auxotrophy of CPSII-deficient mutants, restoring parasite growth and virulence. Our complementation assays reveal that engineered mutations within, or proximal to, the catalytic triad of the N-terminal glutamine amidotransferase (GATase) domain ...
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Order 200ug-Anti-Ornithine Carbamoyl Transferase OCT -polyclonal Antibody 01015884261 at Gentaur 200ug Ornithine Carbamoyl Transferase (OCT)
2-[Benzoyl(carbamoyl)amino]acetic acid | C10H10N2O4 | CID 67704819 - structure, chemical names, physical and chemical properties, classification, patents, literature, biological activities, safety/hazards/toxicity information, supplier lists, and more.
[(2-Cyanoethyl)(phenyl)carbamoyl]methyl 2-(4-methoxyphenoxy)acetate | C20H20N2O5 | CID 2380977 - structure, chemical names, physical and chemical properties, classification, patents, literature, biological activities, safety/hazards/toxicity information, supplier lists, and more.
The unikonts have a triple-gene fusion that is lacking in the bikonts. The three genes that are fused together in the unikonts, but not bacteria or bikonts, encode enzymes for synthesis of the pyrimidine nucleotides: carbamoyl phosphate synthase, dihydroorotase, aspartate carbamoyltransferase. This must have involved a double fusion, a rare pair of events, supporting the shared ancestry of Opisthokonta and Amoebozoa.. Cavalier-Smith[1] originally proposed that unikonts ancestrally had a single flagellum and single basal body. This is unlikely, however, as flagellated opisthokonts, as well as some flagellated Amoebozoa, including Breviata, actually have two basal bodies, as in typical bikonts (even though only one is flagellated in most unikonts). This paired arrangement can also be seen in the organization of centrioles in typical animal cells. In spite of the name of the group, the common ancestor of all unikonts was probably a cell with two basal bodies.. ...
Dagiti unikonta ket addaan iti tallo a panagtitipon ti gene nga awan kadagiti bikonta. Dagiti tallo a genes a naitiptipon kadagiti unikonta ngem saan a ti bakteria wenno dagiti naikodigo nga ensima dagiti bikonta para iti sintesis dagiti pirimidina nukleotido: carbamoyl phosphate synthase, dihydroorotase, aspartate carbamoyltransferase. Daytoy ket mabalin a nakairamanan ti dua a panagtitipon, ti manmano a paris kadagiti pasamak, amangsupsuporat ti nakibinningayan a tinaudan ti Opisthokonta ken Amoebozoa. Ni Cavalier-Smith[4] ket kasisigud a nagisingasing a dagiti unikonta ket nagtaudda nga addaan iti agmaymaysa a flagelo ken agmaymaysa a basal a bagi. Daytoy ket saan a mabalin a kasta, nupay kasta, a kas dagiti adda ti flagelo nga opisthokonta, ken dagiti pay adda ti flagelo nga Amoebozoa, a mairaman ti Breviata, ket pudno nga adda ti dua a basal a bagbagi, a kas dagiti kadawyan a bikonta (urayno maymaysa laeng ti addaan iti flagelo iti kaaduan kadagiti unikonta). Daytoy naparisan a ...
The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. This reaction is the first committed step of the urea cycle, which is important in the removal of excess urea from cells. The encoded protein may also represent a core mitochondrial nucleoid protein. Three transcript variants encoding different isoforms have been found for this gene. The shortest isoform may not be localized to the mitochondrion. Mutations in this gene have been associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent pulmonary hypertension, and susceptibility to venoocclusive disease after bone marrow transplantation.[provided by RefSeq, May 2010 ...
The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. This reaction is the first committed step of the urea cycle, which is important in the removal of excess urea from cells. The encoded protein may also represent a core mitochondrial nucleoid protein. Three transcript variants encoding different isoforms have been found for this gene. The shortest isoform may not be localized to the mitochondrion. Mutations in this gene have been associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent pulmonary hypertension, and susceptibility to venoocclusive disease after bone marrow transplantation.[provided by RefSeq, May 2010]
Order Human OTC Ornithine carbamoyltransferase mitochondrial ELISA Kit 01015848462 at Gentaur OTC(Ornithine carbamoyltransferase, mitochondrial)
Ornithine Carbamoyltransferase Proteins available through Novus Biologicals. Browse our Ornithine Carbamoyltransferase Protein catalog backed by our Guarantee+.
The SCOP classification for the Aspartate carbamoyltransferase, Regulatory-chain, C-terminal domain superfamily including the families contained in it. Additional information provided includes InterPro annotation (if available), Functional annotation, and SUPERFAMILY links to genome assignments, alignments, domain combinations, taxonomic visualisation and hidden Markov model information.
(4-(Benzyl(methyl)carbamoyl)phenyl)boronic acid 874219-49-5 NMR spectrum, (4-(Benzyl(methyl)carbamoyl)phenyl)boronic acid H-NMR spectral analysis, (4-(Benzyl(methyl)carbamoyl)phenyl)boronic acid C-NMR spectral analysis ect.
InterPro provides functional analysis of proteins by classifying them into families and predicting domains and important sites. We combine protein signatures from a number of member databases into a single searchable resource, capitalising on their individual strengths to produce a powerful integrated database and diagnostic tool.
InterPro provides functional analysis of proteins by classifying them into families and predicting domains and important sites. We combine protein signatures from a number of member databases into a single searchable resource, capitalising on their individual strengths to produce a powerful integrated database and diagnostic tool.
Rabbit Polyclonal Anti-Ornithine Carbamoyltransferase Antibody. Validated: IHC, IHC-P. Tested Reactivity: Human, Mouse, Rat. 100% Guaranteed.
The experiments described here reveal the existence of three serendipitous pathways that allow synthesis of PLP in the ΔpdxB strain when any one of seven different genes is overexpressed. The number of genes that allow complementation is surprising; most multicopy suppression experiments reveal fewer genes that can complement a strain lacking a metabolic enzyme. For example, Patrick et al (2007) found that 21 of 104 knockout strains of E. coli could be complemented by multicopy suppression using the ASKA library, but in most cases by only one or two genes. One exception, the ΔglyA strain, was complemented by four genes, one of which encodes an antisigma factor. A second unusual case was described by Miller and Raines (2004, 2005), who found that overexpression of four genes encoding glycokinases with promiscuous glucokinase activity complemented a strain lacking glucokinase. Our finding that seven different genes complement the ΔpdxB strain is, to our knowledge, the record. Furthermore, our ...
Fritz Albert Lipmann (1899 -1986) was awarded half of the 1953 Nobel Prize in Physiology or Medicine for his discovery of coenzyme A and its importance for intermediary metabolism, in which energy is extracted from cellular nutrients and used to build cellular components. His early work included the identification of serine phosphate as the constituent of phosphoproteins, a group of proteins which are chemically bonded to a substance containing phosphoric acid, and an investigation into the Pasteur effect, which showed that oxygen inhibits fermentation in yeast and led to important discoveries on the mechanism of this reaction and on the role of glycolysis in the metabolism of cells in embryos. Lipmanns initial work on coenzyme A led him to investigate the role of phosphorylation in the intermediary reactions of biosynthesis. His later research included demonstrating that carbamoyl phosphate - a molecule involved in clearing the body from excess nitrogen in the urea cycle -- is a carbamoyl ...
CAD antibody (carbamoyl-phosphate synthetase 2, aspartate transcarbamylase, and dihydroorotase) for ICC/IF, WB. Anti-CAD pAb (GTX28406) is tested in Human, Mouse, Rat samples. 100% Ab-Assurance.
A calendar is formed of a plurality of substrates. A first substrate carries indicia thereon which identifies selected time periods, such as days or months of the year. A second substrate is positioned adjacent to the first substrate. The second substrate defines a plurality of cavities dimensioned to individually retain a respective information carrying article, such as a web. Each of the cavities is corresponding supplied with a respective information carrying article. Each indicia on the first substrate is positionally associated with a respective cavity in the second substrate. A third substrate, positioned adjacent to the second substrate, is positioned to retain the information carrying articles releasably within the second substrate. The third substrate provides a rupturable cover over each of the cavities of the second substrate whereby upon the application of a sufficient lateral force on the information carrying article within a selected cavity, the article passes through the cover to a
TY - JOUR. T1 - Phylogenetic Analysis and Protein Modeling of Plasmodium falciparum Aspartate Transcarbamoylase (ATCase). AU - Depamede, Sulaiman. AU - Menz, Ian. PY - 2011. Y1 - 2011. N2 - Unlike most mammalian cells, Plasmodium sp., are unable to utilize preformed pyrimidine bases and nucleosides hence they are reliant solely on de novo pathway. Aspartate transcarbamoylase (ATCase, EC 2.1.3.2) catalyzes the first committed step in de novo pyrimidine biosynthesis pathway, is a potential target for novel anti-parasitic including antimalarial drugs. P. falciparum ATCase has not been studied extensively. To reveal whether it has a regulatory subunit or no and how its evolution, phylogenetic analysis and protein modeling of ATCase P. falciparum were studied. The structural model can be used to identify the possible differences between active sites of mammalian and Plasmodium enzyme. This is important in a relation with antimalarial drug development. Analogous sequences from P. falciparum were ...
NR20R21 (where R20 and R21 are each independently a hydrogen atom or a C1 to C4 alkyl group), a nitro group, a carbamoyl group, an N--(C1 to C4 alkyl)carbamoyl group, an N,N-di(C1 to C4 alkyl)carbamoyl group, or --NHCOR9 (where R9 is a C1 to C4 alkyl group that may be branched), a cyano group, --NR20R21 (where R20 and R21 are each independently a hydrogen atom or a C1 to C4 alkyl group), a nitro group, a carbamoyl group, an N--(C1 to C4 alkyl)carbamoyl group, an N,N-di(C1 to C4 alkyl)carbamoyl group, --NHCOR9 (where R9 is a C1 to C4 alkyl group that may be branched), and a halogen atom; and n is an integer from 1 to 12); (ix) --(CH2)nNR12COR13 (where R12 and R13 are groups independently selected from the group consisting of: (1) a hydrogen atom; (2) a C1 to C4 alkyl group that may be branched; (3) an aryl group, wherein the aryl group may be substituted with at least one group selected from the group consisting of: a C1 to C4 alkyl group that may be branched, a C1 to C5 alkoxy group that may be ...
Carbamoylphosphate synthetase I deficiency (CPS1) Test Cost INR 30000.00 Surat Pune Jaipur Lucknow Kanpur Nagpur Visakhapatnam Indore Thane Bhopal Patna Vadodara Ghaziabad Ludhiana Coimbatore Madurai Meerut Ranchi Allahabad Trivandrum Pondicherry Mysore Aligarh best offer discount price
Carbamoyl phosphate synthetase 1 deficiency (CPS1D) [MIM:237300]: An autosomal recessive disorder of the urea cycle causing hyperammonemia. It can present as a devastating metabolic disease dominated by severe hyperammonemia in neonates or as a more insidious late-onset condition, generally manifesting as life-threatening hyperammonemic crises under catabolic situations. Clinical features include protein intolerance, intermittent ataxia, seizures, lethargy, developmental delay and mental retardation. {ECO:0000269,PubMed:11388595, ECO:0000269,PubMed:11474210, ECO:0000269,PubMed:12655559, ECO:0000269,PubMed:12955727, ECO:0000269,PubMed:15164414, ECO:0000269,PubMed:15617192, ECO:0000269,PubMed:16737834, ECO:0000269,PubMed:17310273, ECO:0000269,PubMed:20578160, ECO:0000269,PubMed:21120950, ECO:0000269,PubMed:22173106, ECO:0000269,PubMed:23649895, ECO:0000269,PubMed:24813853, ECO:0000269,PubMed:26440671, ECO:0000269,PubMed:9711878}. Note=The disease is caused by mutations affecting the gene ...
Ornithine transcarbamylase (OTC) Deficiency information including symptoms, diagnosis, misdiagnosis, treatment, causes, patient stories, videos, forums, prevention, and prognosis.
Ornithine transcarbamylase Ornithine carbamoyltransferase Human OTC trimer. From PDB 1OTH. Available structures: 1c9y, 1ep9, 1fb5, 1fvo, 1oth Identifiers
The worlds first wiki where authorship really matters. Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts.
DI-fusion, le Dépôt institutionnel numérique de lULB, est loutil de référencementde la production scientifique de lULB.Linterface de recherche DI-fusion permet de consulter les publications des chercheurs de lULB et les thèses qui y ont été défendues.
Staphylococcus aureus; pan ID: SAUPAN000811000; symbol: arcB; products: ornithine carbamoyltransferase, ornithine carbamoyltransferase 1, catabolic; orthologs: USA300_FPR3757: SAUSA300_0062 (arcB), USA300_TCH1516: USA300HOU_0067 (arcB1)
This study was designed to determine the possible mechanism by which orotic acid exerts its mitoinhibitory effect on rat hepatocytes in primary culture. Orotic acid inhibited, dose-dependently DNA synthesis in hepatocytes induced by epidermal growth
A process of making a multilayer printed wiring board assembly. The process includes the steps of providing a first and a second substrate made of a dielectric material; depositing a first wiring pattern on the first substrate and a second wiring pattern on the second substrate with a conductive material; depositing a dielectric material on the first and second wiring patterns and defining a via connecting zone on the first and the second wiring pattern for communicating signals between the first and the second wiring pattern by exposing a selective portion of the first and second wiring patterns; depositing a conductive bonding material on the via connecting zone of one of the first and the second wiring pattern; arranging the first and the second substrate in sandwiched juxtaposition such that the via connecting zones of the first and the second wiring pattern are opposite each other and in substantial alignment with each other so that the conductive bonding material deposited on the one of the via
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 ...
Intact rats fed carbamyl phosphate along with a cariogenic diet showed a highly significant reduction in caries. Carbamyl ... The injection of carbamyl phosphate significantly reversed the effect of sucrose-but only in intact rats. Further, carbamyl ... Then it was found that the sucrose effect could be effectively reversed by the infusion of the compound carbamyl phosphate ... an intact hypothalamic-parotid gland endocrine axis was found to be necessary for the effectiveness of carbamyl phosphate. The ...
"Carbamyl Phosphate, the Carbamyl Donor in Enzymatic Citrulline Synthesis1". Journal of the American Chemical Society. 77 (3): ... 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 ...
"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 ...
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 ...
Carrey EA, Campbell DG, Hardie DG (1986). "Phosphorylation and activation of hamster carbamyl phosphate synthetase II by cAMP- ...
Carrey EA, Campbell DG, Hardie DG (Dec 1985). "Phosphorylation and activation of hamster carbamyl phosphate synthetase II by ...
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 ...
"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 ...
Homology of putative targeting sequence to that of carbamyl phosphate synthetase I revealed by correlation of cDNA and protein ...
In ATCase such a transfer is written as Carbamyl phosphate + L-aspertate → {\displaystyle \rightarrow } L-carbamyl aspartate + ... glyceraldehyde 3-phosphate ⇌ {\displaystyle \rightleftharpoons } erythrose 4-phosphate + fructose 6-phosphate. Transfer of acyl ... 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 ...
At present, it is thought that the depletion of the ornithine supply causes the accumulation of carbamyl-phosphate in the urea ...
His research was now focused on aspartate transcarbamylase, which catalyzes the transfer of a carbamyl group from phosphate to ...
... 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. ...
... 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 ...
... 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 ...
Simmer JP, Kelly RE, Scully JL, Evans DR, Rinker Jr AG (1990). "Mammalian carbamyl phosphate synthetase (CPS). DNA sequence and ... 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 ... Clarias batrachus upregulates glutamine synthetase and carbamyl phosphate synthetase III during exposure to high external ...
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 ...
... carbamyl phosphate synthetase (glutamine) glutamine-dependent carbamyl phosphate synthetase carbamoyl phosphate synthetase CPS ... 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 II (EC 6.3.5.5) is an enzyme that catalyzes the reactions that produce carbamoyl phosphate in ...
Homology of putative targeting sequence to that of carbamyl phosphate synthetase I revealed by correlation of cDNA and protein ... glyceraldehyde-3-phosphate dehydrogenase (NAD+) (non-phosphorylating) activity. Cellular component. • extracellular exosome. • ...
... pyridoxal phosphate. Copper AJ, Meister A (1973). "Enzymatic conversion of O-carbamyl-L-serine to pyruvate and ammonia". ...
Clarias batrachus upregulates glutamine synthetase and carbamyl phosphate synthetase III during exposure to high external ... "Role of conserved residues within the carboxy phosphate domain of carbamoyl phosphate synthetase". Biochemistry 35 (45): 14352- ... inactivation of the amidotransferase activity of carbamoyl phosphate synthetase by the antibiotic acivicin ... https://en.wikipedia.org/wiki/Carbamoyl_phosphate_synthetase *↑ Stapleton MA, Javid-Majd F, Harmon MF, Hanks BA, Grahmann JL, ...
... 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.[2] ... wine infected by Brettanomyces yeast in the presence of ammonium phosphate and lysine have also been known to exhibit this ...
  • Carbamyl phosphate synthetase deficiency is listed as a " rare disease " by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). (rightdiagnosis.com)
  • This means that Carbamyl phosphate synthetase deficiency, or a subtype of Carbamyl phosphate synthetase deficiency, affects less than 200,000 people in the US population. (rightdiagnosis.com)
  • A child with carbamyl phosphate synthetase deficiency was studied. (jamanetwork.com)
  • Nonsense-mediated decay of RNA occurs frequently in carbamyl phosphate synthetase I deficiency. (ommbidblog.com)
  • 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, Kohlschütter A, Schäfer 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)
  • Carbamylphosphate synthetase-I deficiency in a newborn: survival after early diagnosis and therapy. (bvsalud.org)
  • 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)
  • To determine whether brief (3 day) treatment with N-carbamylglutamate can improve ureagenesis in adult healthy controls and patients with the following inborn errors of metabolism: N-acetylglutamate synthetase deficiency, propionic acidemia, methylmalonic acidemia, carbamylphosphate synthase deficiency, ornithine transcarbamylase deficiency and the syndrome of hyperammonemia, hypoglycemia and hyperinsulinemia (HHH Syndrome). (clinicaltrials.gov)
  • Condition(s): N-acetylglutamate synthetase deficiency, propionic acidemia, methylmalonic acidemia, carbamylphosphate synthase deficiency, ornithine transcarbamylase deficiency and the syndrome of hyperammonemia, hypoglycemia and hyperinsulinemia. (clinicaltrials.gov)
  • Patients with carbamyl phosphate synthetase and ornithine transcarbamylase deficiency are treated with a low-protein diet, essential amino acids (for neonatal onset disease), caloric supplementation, oral sodium phenylbutyrate (now approved as a prescription drug 11/97), and citrulline or arginine free base. (clinicaltrials.gov)
  • Definition: Carbamoyl phosphate synthetase I deficiency is an autosomal recessive inborn error of metabolism of the urea cycle which causes hyperammonemia. (humpath.com)
  • Two forms of carbamoyl phosphate synthetase deficiency are recognized: a lethal neonatal type and a less severe, delayed-onset type. (humpath.com)
  • Carbamoyl phosphate synthetase I deficiency (CPS) is an inherited condition in which the body is unable to process and remove the waste, ammonia. (babysfirsttest.org)
  • Carbamoyl phosphate synthetase I deficiency (CPS) is estimated to affect one out of every 100,000 to 800,000 babies born worldwide. (babysfirsttest.org)
  • If your baby's newborn screening result for carbamoyl phosphate synthetase I deficiency (CPS) was out of the normal range, your baby's doctor or the state screening program will contact you to arrange for your child to have additional testing. (babysfirsttest.org)
  • There are two main forms of carbomoyl phosphate synthetase I deficiency (CPS), which differ in the age of onset. (babysfirsttest.org)
  • Your baby's doctor may recommend special foods or formulas for children with carbamoyl phosphate synthetase I deficiency (CPS). (babysfirsttest.org)
  • Detection of carbamyl phosphate synthetase 1 deficiency using duodenal biopsy samples. (bmj.com)
  • He is an expert in translational studies of carbamyl phosphate synthetase deficiency and has completed innovative work on nitric oxide in urea cycle and related disorders. (prweb.com)
  • The presence of orotic acid distinguishes ornithine transcarbamylase deficiency from carbamyl phosphate synthetase deficiency. (bmj.com)
  • 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)
  • These include ornithine transcarbamylase deficiency, carbamyl phosphate synthetase deficiency, argininosuccinate synthetase deficiency (citrullinemia), argininosuccinate lyase deficiency and arginase deficiency. (oncologynurseadvisor.com)
  • Molecular characterization of carbamoyl-phosphate synthetase (CPS1) deficiency using human recombinant CPS1 as a key tool. (semanticscholar.org)
  • Understanding carbamoyl-phosphate synthetase I (CPS1) deficiency by using expression studies and structure-based analysis. (semanticscholar.org)
  • The enzyme is compared with carbamyl phosphate synthetases of other organisms. (asm.org)
  • The decreased plasma concentrations of urea during supplementation of rbST might be due to the effect of bovine somatotropin via N-actyl glutamate, on carbamyl phophate synthetase, a key enzyme in the urea cycle (Oddy and Lindsay, 1986). (thefreedictionary.com)
  • [1] This enzyme catalyzes the reaction of ATP and bicarbonate to produce carboxy phosphate and ADP . (wikipedia.org)
  • 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)
  • 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 II (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)
  • Genetic variation in the mitochondrial enzyme carbamyl-phosphate synthetase I predisposes children to increased pulmonary artery pressure following surgical repair of congenital heart defects: a validated genetic association study. (nih.gov)
  • 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)
  • 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)
  • at this step, N-acetylglutamate exerts its regulatory control on the mediating enzyme, carbamyl phosphate synthetase (CPS). (medscape.com)
  • A mutated CPS1 gene may result in a carbamoyl phosphate synthetase I enzyme that is shorter than normal or the wrong shape, or may prevent the enzyme from being produced at all. (nih.gov)
  • If the carbamoyl phosphate synthetase enzyme is misshapen or missing, it cannot fulfill its role in the urea cycle. (nih.gov)
  • Carbamoyl phosphate synthetase I is a ligase enzyme located in the mitochondria involved in the production of urea. (wikidoc.org)
  • The resulting molecule of carbamoyl phosphate leaves the enzyme. (wikidoc.org)
  • 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). (nih.gov)
  • Amino acid sequencing of the 150-kDa band revealed sequence identity to carbamyl phosphate synthetase I (CPS I) and a high degree of homology to the related trifunctional enzyme known as CAD (for carbamyl phosphate synthetase, aspartate transcarbamylase, and dihydroorotase). (unl.edu)
  • 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)
  • 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)
  • Its synthesis is initiated by the formation of carbamoyl phosphate (CP) in the cytoplasm, with ammonia derived from glutamine. (biomedsearch.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)
  • Two moles of ATP are utilized for the synthesis of one molecule of carbamyl phosphate, making the reaction essentially irreversible. (kegg.jp)
  • 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. (wikidoc.org)
  • The hyperammonemia observed in methylmalonic acidemia is thought to arise because accumulated propionyl CoA interferes with formation of N-acetylglutamate, an obligatory activator of carbamyl phosphate synthase, the initial step in urea synthesis (4). (thefreelibrary.com)
  • Funghini S, Donati MA, Pasquini E, Zammarchi E, Morrone A. Structural organization of the human carbamyl phosphate synthetase I gene (CPS1) and identification of two novel genetic lesions. (nih.gov)
  • Etiology mutation in gene encoding carbamoyl phosphate synthetase I (CPS1) (MIM.608307). (humpath.com)
  • 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. (wikidoc.org)
  • The accumulation of carbamyl phosphate, which is associated with inherited defects of the urea cycle, stimulates the pyrimidine de novo pathway, leading to increased production of orotate. (thefreedictionary.com)
  • There are three different forms that serve very different functions: Carbamoyl phosphate synthetase I (mitochondria, urea cycle) Carbamoyl phosphate synthetase II (cytosol, pyrimidine metabolism). (wikipedia.org)
  • Compounds comprising the urea cycle are numbered sequentially, beginning with carbamyl phosphate (1). (medscape.com)
  • Carbamoyl phosphate is then put into the urea cycle to eventually create urea. (wikidoc.org)
  • Carboxy phosphate reacts with ammonia to give carbamic acid . (wikipedia.org)
  • The carboxylphosphate then reacts with ammonia to form carbamic acid, releasing inorganic phosphate. (wikipedia.org)
  • The small subunit contains the glutamine binding site and catalyses the hydrolysis of glutamine to glutamate and ammonia , which is in turn used by the large chain to synthesize carbamoyl phosphate. (wikipedia.org)
  • Inhibition of carbamoyl-phosphate synthase (ammonia) by Tris and Hepes. (wikipedia.org)
  • Carbamoyl-phosphate synthetase (CPS) contains a small amidotransferase subunit (GLN) that hydrolyzes glutamine and transfers ammonia to the large synthetase subunit (SYN), where CP biosynthesis occurs in the presence of ATP and CO 2 . (asm.org)
  • The critical step in this interference with ammonia metabolism was the inhibition of carbamyl phosphate synthetase. (aspetjournals.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. (wikidoc.org)
  • the first waste nitrogen molecule derives from free ammonia in the carbamyl phosphate synthetase (CPS) reaction. (love-pet.net)
  • A second molecule of ATP then phosphorylates carbamic acid, creating carbamoyl phosphate. (wikipedia.org)
  • The structure of carbamoyl phosphate synthetase determined to 2.1 A resolution. (ebi.ac.uk)
  • Structure of carbamoyl phosphate synthetase: a journey of 96 A from substrate to product. (semanticscholar.org)
  • Carbamyl phosphate is the intermediate in the reaction and its biosynthesis in the earthworm is mediated by a carbamyl phosphate synthetase system similar to that of ureotelic vertebrates. (sciencemag.org)
  • Evidence that carboxyphosphate is a kinetically competent intermediate in the carbamyl phosphate synthetase reaction. (semanticscholar.org)
  • Catalysis of the reaction: 2 ATP + L-glutamine + CO2 + H2O = 2 ADP + phosphate + glutamate + carbamoyl phosphate. (yeastgenome.org)
  • The large subunit contains two active sites, one for the production of carboxyphosphate, and the other for the production of carbamoyl phosphate. (wikidoc.org)
  • Cloning, expression, and functional interactions of the amidotransferase domain of mammalian CAD carbamyl phosphate synthetase. (ebi.ac.uk)
  • 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)
  • [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)
  • Liver glutamine synthetase (GS), carbamoyl phosphate synthetase III (CPS), ornithine carbamoyl transferase (OCT) and arginase (ARG) activities were also measured. (biologists.org)
  • In liver tissue, glutamine synthetase is mitochondrial where it functions to provide glutamine to carbamyl phosphate synthetase-III for urea biosynthesis. (rice.edu)
  • Carbamoyl phosphate (CP) is an intermediate in pyrimidine and arginine biosynthesis. (asm.org)
  • Thus, carbamyl phosphate is a high-energy phosphate, can be formed from inorganic materials under very mild conditions, and is used in the biosynthesis of components of proteins and nucleic acids. (google.co.uk)
  • Chemical Basis of the carbomi dodiacetyl micro-method for estimation of urea, citrulline and carbamyl derivatives. (thefreedictionary.com)
  • Veniamin HP, Vakirtzi-Lemonias C, Chemical basis of the carbomido-diacetyl, micro-method for estimation of urea, citrulline and carbamyl derivatives, Clin. (thefreedictionary.com)
  • In the present study, we determined possible associations between MIF levels, hemodynamic and histological parameters, and mitochondrial carbamyl-phosphate synthetase I (CPSI) T1405N polymorphism in a similar population. (cdc.gov)
  • 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)
  • Carbamylation of glutamate dehydrogenase and other mitochondrial proteins by biosynthetic carbamyl phosphate. (thefreedictionary.com)
  • Inactivation of bovine glutamate dehydrogenase by carbamyl phosphate and cyanate. (thefreedictionary.com)
  • Carbamoyl-phosphate synthetase (glutamine-hydrolysing) is also known as: hydrogen-carbonate:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating) carbamyl phosphate synthetase (glutamine) glutamine-dependent carbamyl phosphate synthetase carbamoyl phosphate synthetase CPS carbon-dioxide:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating) Anderson PM, Meister A (December 1965). (wikipedia.org)
  • Structural defects within the carbamate tunnel of carbamoyl phosphate synthetase. (ebi.ac.uk)
  • and the phosphorylation of carbamate forming carbamoyl phosphate. (kegg.jp)
  • Carbamoyl phosphate synthetase II (cytosol, pyrimidine metabolism ). (wikipedia.org)
  • Four of these five diseasesâ€"deficiencies of carbamyl phosphate synthetase (CPS) (OMIM 237300), ornithine transcarbamylase (OTC) (OMIM 311250), argininosuccinic acid synthetase (AS) (OMIM 215700), and argininosuccinate lyase (AL) (OMIM 207900)â€"are characterized by signs and symptoms induced by the accumulation of precursors of urea, principally ammonium and glutamine. (ommbidblog.com)
  • Carbamoyl phosphate synthetase: closure of the B-domain as a result of nucleotide binding. (semanticscholar.org)
  • Sequential up-regulation of thyroid hormone b receptor, ornithine transcarbamylase and carbamyl phosphate synthetase mRNAs in the liver of Rana catesbeiana tadpoles during spontaneous and thyroid hormone-induced metamorphosis. (thefreedictionary.com)
  • In case 1 hepatic ornithine transcarbamylase and carbamyl phosphate synthetase activities were within normal limits. (bmj.com)
  • Enzymes in the transcarbamylase family catalyze the transfer of a carbamyl group from carbamyl phosphate (CP) to an amino group of a second substrate. (mdpi.com)
  • Gene structure of human carbamylphosphate synthetase 1 and novel mutations in patients with neonatal onset. (nih.gov)
  • Carbamoyl phosphate synthetase polymorphisms as a risk factor for necrotizing enterocolitis. (nih.gov)
  • HFE C282Y is a risk factor for hepatic veno-occlusive disease (HVOD) and carbamyl-phosphate synthetase (CPS) polymorphisms may counteract its adverse effects. (irondisorders.org)
  • The rate-limiting step is carbamoyl phosphate synthetase (CPS) disposal of waste nitrogen. (medscape.com)
  • Biopsy of liver tissue revealed that the level of mitochondrial carbamoyl phosphate synthetase was about 15-25% that of normal. (love-pet.net)
  • Urea, reported by Nalbandian (1971) and by McCurdy (1971), cyanate, reported by Cerami and Manning (1971), and carbamyl phosphate (carbamyl P), reported by Kraus and Kraus (1971) have been successfully used to inhibit the sickling phenomenon. (springer.com)
  • Urea is proposed to act reversibly by interfering with the hydrophobic bonding of hemoglobin molecules, (Murayama, 1971), while cyanate is reported to act irreversibly through the carbamyl ation of the amino terminal valines, (Cerami and Manning, 1971). (springer.com)
  • Protonation of cyanate leads to the formation of isocyanic acid, which reacts with the a and e amino groups on proteins, forming a carbamyl moiety (37). (thefreedictionary.com)
  • The isolation of cytidine diphosphate glycerol, cytidine diphosphate ribitol and mannitol 1-phosphate from Lactobacillus arabinosus. (springer.com)
  • In turn, carbamic acid reacts with a second ATP to give carbamoyl phosphate plus ADP . (wikipedia.org)

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