Detailed account or statement or formal record of data resulting from empirical inquiry.
Activities associated with the disposition of the dead. It excludes cultural practices such as funeral rites.
A loose confederation of computer communication networks around the world. The networks that make up the Internet are connected through several backbone networks. The Internet grew out of the US Government ARPAnet project and was designed to facilitate information exchange.
The portion of an interactive computer program that issues messages to and receives commands from a user.
Databases containing information about PROTEINS such as AMINO ACID SEQUENCE; PROTEIN CONFORMATION; and other properties.
Sequential operating programs and data which instruct the functioning of a digital computer.
The addition of descriptive information about the function or structure of a molecular sequence to its MOLECULAR SEQUENCE DATA record.
Rare congenital metabolism disorders of the urea cycle. The disorders are due to mutations that result in complete (neonatal onset) or partial (childhood or adult onset) inactivity of an enzyme, involved in the urea cycle. Neonatal onset results in clinical features that include irritability, vomiting, lethargy, seizures, NEONATAL HYPOTONIA; RESPIRATORY ALKALOSIS; HYPERAMMONEMIA; coma, and death. Survivors of the neonatal onset and childhood/adult onset disorders share common risks for ENCEPHALOPATHIES, METABOLIC, INBORN; and RESPIRATORY ALKALOSIS due to HYPERAMMONEMIA.
Elevated level of AMMONIA in the blood. It is a sign of defective CATABOLISM of AMINO ACIDS or ammonia to UREA.
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)
Errors in metabolic processes resulting from inborn genetic mutations that are inherited or acquired in utero.
A rare autosomal recessive disorder of the urea cycle. It is caused by a deficiency of the hepatic enzyme ARGINASE. Arginine is elevated in the blood and cerebrospinal fluid, and periodic HYPERAMMONEMIA may occur. Disease onset is usually in infancy or early childhood. Clinical manifestations include seizures, microcephaly, progressive mental impairment, hypotonia, ataxia, spastic diplegia, and quadriparesis. (From Hum Genet 1993 Mar;91(1):1-5; Menkes, Textbook of Child Neurology, 5th ed, p51)
The sodium salt of BENZOIC ACID. It is used as an antifungal preservative in pharmaceutical preparations and foods. It may also be used as a test for liver function.
Rare autosomal recessive disorder of the urea cycle which leads to the accumulation of argininosuccinic acid in body fluids and severe HYPERAMMONEMIA. Clinical features of the neonatal onset of the disorder include poor feeding, vomiting, lethargy, seizures, tachypnea, coma, and death. Later onset results in milder set of clinical features including vomiting, failure to thrive, irritability, behavioral problems, or psychomotor retardation. Mutations in the ARGININOSUCCINATE LYASE gene cause the disorder.
An element with the atomic symbol N, atomic number 7, and atomic weight [14.00643; 14.00728]. Nitrogen exists as a diatomic gas and makes up about 78% of the earth's atmosphere by volume. It is a constituent of proteins and nucleic acids and found in all living cells.
Flagellate EUKARYOTES, found mainly in the oceans. They are characterized by the presence of transverse and longitudinal flagella which propel the organisms in a rotating manner through the water. Dinoflagellida were formerly members of the class Phytomastigophorea under the old five kingdom paradigm.
An algal bloom where the algae produce powerful toxins that can kill fish, birds, and mammals, and ultimately cause illness in humans. The harmful bloom can also cause oxygen depletion in the water due to the death and decomposition of non-toxic algae species.
Toxic or poisonous substances elaborated by marine flora or fauna. They include also specific, characterized poisons or toxins for which there is no more specific heading, like those from poisonous FISHES.
Compounds based on an 8-membered heterocyclic ring including an oxygen. They can be considered medium ring ethers.
Inorganic compounds that contain nitrogen as an integral part of the molecule.
Highly toxic compound which can cause skin irritation and sensitization. It is used in manufacture of azo dyes.
A naphthalene derivative with carcinogenic action.
Hydrobromic acid (HBr). A solution of hydrogen bromide gas in water.
Salts of nitrous acid or compounds containing the group NO2-. The inorganic nitrites of the type MNO2 (where M=metal) are all insoluble, except the alkali nitrites. The organic nitrites may be isomeric, but not identical with the corresponding nitro compounds. (Grant & Hackh's Chemical Dictionary, 5th ed)
A genus known for fibers obtained from their leaves: sisal from A. sisalana, henequen from A. fourcroyoides and A. cantala, or Manila-Maguey fiber from A. cantala. Some species provide a sap that is fermented to an intoxicating drink, called pulque in Mexico. Some contain agavesides.
A strong corrosive acid that is commonly used as a laboratory reagent. It is formed by dissolving hydrogen chloride in water. GASTRIC ACID is the hydrochloric acid component of GASTRIC JUICE.
Any liquid or solid preparation made specifically for the growth, storage, or transport of microorganisms or other types of cells. The variety of media that exist allow for the culturing of specific microorganisms and cell types, such as differential media, selective media, test media, and defined media. Solid media consist of liquid media that have been solidified with an agent such as AGAR or GELATIN.
Amino acids and chains of amino acids connected by peptide linkages.
Errors in the metabolism of LIPIDS resulting from inborn genetic MUTATIONS that are heritable.
A heterogeneous group of inherited metabolic disorders marked by absent or dysfunctional PEROXISOMES. Peroxisomal enzymatic abnormalities may be single or multiple. Biosynthetic peroxisomal pathways are compromised, including the ability to synthesize ether lipids and to oxidize long-chain fatty acid precursors. Diseases in this category include ZELLWEGER SYNDROME; INFANTILE REFSUM DISEASE; rhizomelic chondrodysplasia (CHONDRODYSPLASIA PUNCTATA, RHIZOMELIC); hyperpipecolic acidemia; neonatal adrenoleukodystrophy; and ADRENOLEUKODYSTROPHY (X-linked). Neurologic dysfunction is a prominent feature of most peroxisomal disorders.
A flavoprotein oxidoreductase that has specificity for long-chain fatty acids. It forms a complex with ELECTRON-TRANSFERRING FLAVOPROTEINS and conveys reducing equivalents to UBIQUINONE.
A flavoprotein oxidoreductase that has specificity for medium-chain fatty acids. It forms a complex with ELECTRON TRANSFERRING FLAVOPROTEINS and conveys reducing equivalents to UBIQUINONE.
The ordered rearrangement of gene regions by DNA recombination such as that which occurs normally during development.
A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal GLUCAGON-LIKE PEPTIDES. Glucagon is secreted by PANCREATIC ALPHA CELLS and plays an important role in regulation of BLOOD GLUCOSE concentration, ketone metabolism, and several other biochemical and physiological processes. (From Gilman et al., Goodman and Gilman's The Pharmacological Basis of Therapeutics, 9th ed, p1511)
An anti-inflammatory 9-fluoro-glucocorticoid.
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 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.
An enzyme that catalyzes the formation of carbamoyl phosphate from ATP, carbon dioxide, and ammonia. This enzyme is specific for arginine biosynthesis or the urea cycle. Absence or lack of this enzyme may cause CARBAMOYL-PHOSPHATE SYNTHASE I DEFICIENCY DISEASE. EC 6.3.4.16.
A 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.
NATIONAL LIBRARY OF MEDICINE service for health professionals and consumers. It links extensive information from the National Institutes of Health and other reviewed sources of information on specific diseases and conditions.
Longitudinal patient-maintained records of individual health history and tools that allow individual control of access.

Hepatocyte gene therapy in a large animal: a neonatal bovine model of citrullinemia. (1/173)

The development of gene-replacement therapy for inborn errors of metabolism has been hindered by the limited number of suitable large-animal models of these diseases and by inadequate methods of assessing the efficacy of treatment. Such methods should provide sensitive detection of expression in vivo and should be unaffected by concurrent pharmacologic and dietary regimens. We present the results of studies in a neonatal bovine model of citrullinemia, an inborn error of urea-cycle metabolism characterized by deficiency of argininosuccinate synthetase and consequent life-threatening hyperammonemia. Measurements of the flux of nitrogen from orally administered 15NH4 to [15N]urea were used to determine urea-cycle activity in vivo. In control animals, these isotopic measurements proved to be unaffected by pharmacologic treatments. Systemic administration of a first-generation E1-deleted adenoviral vector expressing human argininosuccinate synthetase resulted in transduction of hepatocytes and partial correction of the enzyme defect. The isotopic method showed significant restoration of urea synthesis. Moreover, the calves showed clinical improvement and normalization of plasma glutamine levels after treatment. The results show the clinical efficacy of treating a large-animal model of an inborn error of hepatocyte metabolism in conjunction with a method for sensitively measuring correction in vivo. These studies will be applicable to human trials of the treatment of this disorder and other related urea-cycle disorders.  (+info)

Metabolic capacity for L-citrulline synthesis from ammonia in rat isolated colonocytes. (2/173)

Ammonia is present at high concentration in the colon lumen and is considered a colon cancer suspect. Furthermore, ammonia usually eliminated by the liver in the ornithine cycle is considered highly toxic to cerebral function when present in excess in the blood plasma. Therefore, the metabolic pathways involved in ammonia metabolism in colonocytes were studied in the present study. Rat colonocytes were found equipped with low carbamoylphosphate synthase I activity, high ornithine carbamoyltransferase and arginase activities and low argininosuccinate synthase activity. High (10 and 50 mmol/l) NH4Cl concentrations but not low concentrations (1 and 5 mmol/l) were found able to increase respectively 3- and 10-fold the conversion of radioactive L-arginine to L-citrulline. In contrast, very low capacity for L-citrulline conversion to L-arginine is found in colonocytes. It is concluded that an incomplete ornithine cycle is operative in colonocytes which results in ammonia stimulated L-citrulline production. The contribution of this metabolic pathway in relation to ammonia detoxication by colonocytes is discussed.  (+info)

An adult-onset case of argininosuccinate synthetase deficiency presenting with atypical citrullinemia. (3/173)

A 52-year-old heavy drinker presented with repeated episodes of disturbance of consciousness and an increase in serum ammonia level, triggered by excessive alcohol intake. He was diagnosed as having adult-onset citrullinemia with deficiency of hepatic argininosuccinate synthetase (ASS) activity. Cranial magnetic resonance imaging (MRI) showed high-intensity lesions in the central pons and the bilateral middle cerebellar peduncles on T2-weighted images. Although almost all cases of adult-onset citrullinemia have been reported to be enzymologically classified as type II, the serum amino acid pattern and serum level of human pancreatic secretory trypsin inhibitor (hPSTI) were atypical for type II in the present case.  (+info)

Induction of argininosuccinate synthetase in rat brain glial cells after striatal microinjection of immunostimulants. (4/173)

The enzyme argininosuccinate synthetase (ASS) initiates the metabolic pathway leading from L-citrulline to L-arginine, the only physiological substrate of all isoforms of nitric oxide synthases. The presence of ASS in glial cells in vivo was investigated by immunohistochemical methods in a model of rat brain inflammation. Phosphate-buffered saline or a mixture of bacterial lipopolysaccharide and interferon-gamma was injected into the left striatum, and animals were killed 24 hours later. Ipsilateral and contralateral sides of brain sections were incubated with an antiserum against ASS or antibodies against cell-specific markers. In the three areas examined, striatum, corpus callosum, and cortex, a strong induction of ASS immunoreactivity was observed in glial cells after injection of immunostimulants. A detailed quantitative analysis of double-stained sections revealed that ASS was almost exclusively expressed in reactive, ED1-positive microglial cells/brain macrophages in immunostimulant- or sham-injected ipsilateral sides of the sections. Furthermore, ASS/ED1 costaining was observed in perivascular cells. Colocalization of ASS with astroglial marker glial fibrillary acidic protein was given only occasionally after immunostimulation. ASS-positive neurons were detected in control and experimental animals; staining intensity was comparable in both cases. The results suggest that neurons express ASS constitutively, whereas the enzyme is induced in glial cells in response to proinflammatory stimuli. This finding is the first demonstration of an induction of a pathway auxiliary to generation of nitric oxide in brain in response to immunostimulants and provides new insight into neural arginine metabolism.  (+info)

Using genomic information to investigate the function of ThiI, an enzyme shared between thiamin and 4-thiouridine biosynthesis. (5/173)

The gene thiI encodes a protein (ThiI) that plays a role in the transfer of sulfur from cysteine to both thiamin and 4-thiouridine, but the reaction catalyzed by ThiI remains undetermined. Based upon sequence alignments, ThiI shares a unique "P-loop" motif with the PPi synthetase family, four enzymes that catalyze adenylation and subsequent substitution of carbonyl oxygens. To test whether or not this motif is critical for ThiI function, the Asp in the motif was converted to Ala (D189A), and a screen for in vivo 4-thiouridine production revealed the altered enzyme to be inactive. Further scrutiny of sequence data and the crystal structures of two members of the PPi synthetase family implicated Lys321 in the proposed adenylation function of ThiI, and the critical nature of Lys321 has been demonstrated by site-directed mutagenesis and genetic screening. Our results, then, indicate that ThiI catalyzes the adenylation of a substrate at the expense of ATP, a narrowing of possible reactions that provides a strong new basis for deducing the early steps in the transfer of sulfur from cysteine to both thiamin and 4-thiouridine.  (+info)

Augmentation of urea-synthetic capacity by inhibition of nitric oxide synthesis in butyrate-induced differentiated human hepatocytes. (6/173)

We have recently developed an in vitro differentiation model of immortalized non-transformed human hepatocytes using butyrate, and observed the induction of inducible NO synthase (iNOS). In this study, we analyzed the effect of NO on the urea-synthetic capacity of these cells. The inhibition of iNOS during butyrate treatment significantly increased the urea-synthetic capacity as compared to that of butyrate treatment alone, possibly through the further induction of ornithine transcarbamylase expression. Therefore, the inhibition of NO production might be useful for obtaining more differentiated hepatocytes in the process of in vitro induction of hepatocyte-specific differentiation.  (+info)

Regulation of diaphragmatic nitric oxide synthase expression during hypobaric hypoxia. (7/173)

Nitric oxide (NO) is normally synthesized inside skeletal muscle fibers by both endothelial (eNOS) and neuronal (nNOS) nitric oxide synthases. In this study, we evaluated the influence of hypobaric hypoxia on the expression of NOS isoforms, argininosuccinate synthetase (AS), argininosuccinate lyase (AL), and manganese superoxide dismutase (Mn SOD) in the ventilatory muscles. Rats were exposed to hypobaric hypoxia ( approximately 95 mmHg) from birth for 60 days or 9-11 mo. Age-matched control groups of rats also were examined. Sixty days of hypoxia elicited approximately two- and ninefold increases in diaphragmatic eNOS and nNOS protein expression (evaluated by immunoblotting), respectively, and about a 50% rise in diaphragmatic NOS activity. In contrast, NOS activity and the expression of these proteins declined significantly in response to 9 mo of hypoxia. Hypoxia elicited no significant alterations in AS, AL and Mn SOD protein expression. Moreover, the inducible NOS (iNOS) was not detected in normoxic and hypoxic diaphragmatic samples. We conclude that diaphragmatic NOS expression and activity undergo significant adaptations to hypobaric hypoxia and that iNOS does not participate in this response.  (+info)

Mutation analysis of Korean patients with citrullinemia. (8/173)

Citrullinemia is an autosomal recessive disease due to the mutations in the argininosuccinate synthetase (ASS) gene. Mutation analysis was performed on three Korean patients with citrullinemia. All of the three patients had the splicing mutation previously reported as IVS6-2A>G mutation. Two had Gly324Ser mutation and the other patient had a 67-bp insertion mutation in exon 15. The IVS6-2A>G mutation was reported to be found frequently in Japanese patients with citrullinemia, but Caucasian patients showed the extreme mutational heterogeneity. Although a limited number of Korean patients were studied, the IVS6-2A>G mutation appears to be one of the most frequent mutant alleles in Korean patients with citrullinemia. The Gly324Ser mutation identified in two patients also suggests the possible high frequency of this mutation in Korean patients as well.  (+info)

Citrullinemia type I is an autosomal recessive disorder that is caused by a deficiency of the urea cycle enzyme argininosuccinate synthetase (ASS1). Deficiency of ASS1 shows various clinical manifestations encompassing severely affected patients with fatal neonatal hyperammonemia as well as asymptomatic individuals with only a biochemical phenotype. This is a comprehensive report of all 87 mutations found to date in the ASS1 gene on chromosome 9q34.1. A large proportion of the mutations (n=27) are described here for the first time. Mutations are distributed throughout exons 3 to 15, most of them being identified in exons 5, 12, 13, and 14. The mutation G390R in exon 15 is the single most common mutation in patients with the classical phenotype. Certain mutations clearly link to specific clinical courses but the clinical phenotype cannot be anticipated in all patients. This update presents a survey of the correlation between mutations in the ASS1 gene and the respective clinical courses as ...
TY - JOUR. T1 - Targeting argininosuccinate synthetase negative melanomas using combination of arginine degrading enzyme and cisplatin. AU - Savaraj, Niramol. AU - Wu, Chunjing. AU - Li, Ying Ying. AU - Wangpaichitr, Medhi. AU - You, Min. AU - Bomalaski, John. AU - He, Wei. AU - Kuo, Macus Tien. AU - Feun, Lynn G. PY - 2015. Y1 - 2015. N2 - Loss of argininosuccinate synthetase (ASS) expression in melanoma makes these tumor cells vulnerable to arginine deprivation. Pegylated arginine deiminase (ADIPEG20) which degrades arginine to citrulline and ammonia has been used clinically and partial responses and stable disease have been noted with minimal toxicity. In order to improve the therapeutic efficacy of ADI-PEG20, we have combined ADI-PEG20 with a DNA damaging agent, cisplatin. We have shown that the combination of the two drugs together significantly improved the therapeutic efficacy when compared to ADI-PEG20 alone or cisplatin alone in 4 melanoma cell lines, regardless of their BRAF mutation. ...
Although accumulating evidence highlights the importance of p53-mediated metabolism in tumor suppression (2, 47), the mechanisms by which p53 drives dynamic nutrient status in harmony with canonical p53 functions remain poorly understood. Here, we show that p53 activates the penultimate step of de novo arginine synthesis pathway through the direct induction of the rate-limiting enzyme ASS1. Furthermore, we demonstrate that ASS1 deficiency induced anomalous Akt phosphorylation, resulting in rendering cells more susceptible to genotoxic stress.. Although we have demonstrated that p53 drives the de novo arginine synthesis pathway via ASS1 induction under genotoxic conditions, argininosuccinate lyase (ASL), which directly produces arginine from argininosuccinate, was not induced by p53 in HCT116 cells. These results suggest that ASS1 is the sole node connecting p53 to the de novo arginine synthesis pathway. Because ASS1 is a rate-limiting enzyme of the de novo arginine synthesis pathway, ASS1 ...
Argininosuccinate synthetase (ASS), an integral enzyme to synthesize arginine is usually down regulated in many tumors including hepatocellular carcinoma (HCC). only occurs in SNU398 and SNU387, and not in HepG2 and Huh-1 (ASS(+)) cells, purchase EPZ-5676 and it is partly because of reduced anti-apoptotic protein X-linked inhibitor of apoptosis proteins (XIAP), myeloid leukemia cell differentiation proteins (Mcl-1) and B-cell lymphoma-2 (Bcl-2). Significantly, insufficient ASS also affects important enzymes in pyrimidine synthesis (carbamoyl-phosphate synthetase2, aspartate transcarbamylase and dihydrooratase (CAD) and thymidylate synthase (TS)) and malate dehydrogenase-1 (MDH-1) in TCA routine. ADI-PEG20 treatment reduced these enzymes and produced them more purchase EPZ-5676 susceptible to 5-FU. Transfection of ASS restored these enzymes and abolished the awareness to mixture and ADI-PEG20 treatment. General, our data claim that ASS affects multiple enzymes involved with 5-FU sensitivity. ...
L-citrulline plays an important role in human health and nutrition and is an intermediate of the L-arginine biosynthetic pathway. L-citrulline is a by-product of L-arginine production by Corynebacterium glutamicum. In this study, C. glutamicum was engineered for overproduction of L-citrulline as major product without L-arginine being produced as by-product. To this end, L-arginine biosynthesis was derepressed by deletion of the arginine repressor gene argR and conversion of L-citrulline towards L-arginine was avoided by deletion of the argininosuccinate synthetase gene argG. Moreover, to facilitate L-citrulline production the gene encoding a feedback resistant N-acetyl L-glutamate kinase argBfbr as well as the gene encoding L-ornithine carbamoylphosphate transferase argF were overexpressed. The resulting strain accumulated 44.1 ± 0.5 mM L-citrulline from glucose minimal medium with a yield of 0.38 ± 0.01 g⋅g−1 and a volumetric productivity of 0.32 ± 0.01 g⋅l−1⋅h−1. In addition, production
Many melanomas do not express argininosuccinate synthetase (ASS) which is a key enzyme in the process of arginine biosynthesis. As a result, these melanoma cells need exogenous arginine supply for survival and proliferation while normal cell do not. A new drug targeting this metabolic defect of melanoma has been developed by Polaris, Inc. (recombinant arginine deiminase conjugated with polyethylene glycol-20 or ADI-PEG20). This complex can effectively degrade blood arginine to citrulline. Phase I/II clinical trials with ADI-PEG20 have shown promising activity in patients with advanced melanoma. However, our laboratory studies have demonstrated that after treatment with ADI-PEG20, some melanoma cells are able to induce the production of ASS, while others have the ability to undergo prolonged autophagy as well as low requirement for arginine to survive. Thus, arginine deprivation results in growth inhibition without cell death. On the other hand, TRAIL has been used to induce apoptosis in certain ...
Pancreatic cancer is a leading cause of cancer-related deaths in the world with a 5-year survival rate of less than 6%. Currently, there is no successful therapeutic strategy for advanced pancreatic cancer, and new effective strategies are urgently needed. Recently, an arginine deprivation agent, arginine deiminase, was found to inhibit the growth of some tumor cells (i.e., hepatocellular carcinoma, melanoma, and lung cancer) deficient in argininosuccinate synthetase (ASS), an enzyme used to synthesize arginine. The purpose of this study was to evaluate the therapeutic efficacy of arginine deiminase in combination with gemcitabine, the first line chemotherapeutic drug for patients with pancreatic cancer, and to identify the mechanisms associated with its anticancer effects. In this study, we first analyzed the expression levels of ASS in pancreatic cancer cell lines and tumor tissues using immunohistochemistry and RT-PCR. We further tested the effects of the combination regimen of arginine deiminase
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TY - JOUR. T1 - Arginine starvation kills tumor cells through aspartate exhaustion and mitochondrial dysfunction. AU - Cheng, Chun Ting. AU - Qi, Yue. AU - Wang, Yi Chang. AU - Chi, Kevin K.. AU - Chung, Yiyin. AU - Ouyang, Ching. AU - Chen, Yun Ru. AU - Oh, Myung Eun. AU - Sheng, Xiangpeng. AU - Tang, Yulong. AU - Liu, Yun Ru. AU - Lin, H. Helen. AU - Kuo, Ching Ying. AU - Schones, Dustin. AU - Vidal, Christina M.. AU - Chu, Jenny C.Y.. AU - Wang, Hung Jung. AU - Chen, Yu Han. AU - Miller, Kyle M.. AU - Chu, Peiguo. AU - Yen, Yun. AU - Jiang, Lei. AU - Kung, Hsing-Jien. AU - Ann, David K.. PY - 2018/12/1. Y1 - 2018/12/1. N2 - Defective arginine synthesis, due to the silencing of argininosuccinate synthase 1 (ASS1), is a common metabolic vulnerability in cancer, known as arginine auxotrophy. Understanding how arginine depletion kills arginine-auxotrophic cancer cells will facilitate the development of anti-cancer therapeutic strategies. Here we show that depletion of extracellular arginine in ...
1KH2: Crystal structure of argininosuccinate synthetase from Thermus thermophilus HB8. Structural basis for the catalytic action.
Proc Natl Acad Sci U S A. 2013 Dec 17;110(51):E5006-15. doi: 10.1073/pnas.1321305110. Epub 2013 Dec 2. Research Support, N.I.H., Extramural
TY - JOUR. T1 - Arginine deprivation and tumour cell death: arginase and its inhibition. AU - Wheatley, Denis. AU - Philip, R.. AU - Campbell, E.. PY - 2003. Y1 - 2003. N2 - Arginase treatment of cell cultures reduced arginine in the medium to similar to micromolar levels within 5-30 min, and proved as effective as arginine-free medium (AFM) prepared by formulation. The enzyme was heat stable and as active at pH 7.2 as at pH 9.9. It persisted in culture for at least 3 days with only a small diminution in its speed of action, and still actively destroyed arginine after 6 days, since arginine supplementation failed to rescue viable cells.Addition of L-norvaline, an inhibitor of arginase, rescued cells from arginase-induced deprivation. Its efficacy at low concentrations was short-lived (probably , 1 day), while at higher concentrations it did not appear to inhibit completely the enzyme. However, L-norvaline at these same levels also slowed the growth of positive non-enzyme treated controls ...
Complete information for ASS1P14 gene (Pseudogene), Argininosuccinate Synthetase 1 Pseudogene 14, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
Complete information for ASS1P12 gene (Pseudogene), Argininosuccinate Synthetase 1 Pseudogene 12, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
... SAN DIEGO Dec. 2 2013 /- ... This is another IND that expands the portfolio of indications for whi...About ADI-PEG 20 ...ADI-PEG 20 is a biologic being developed by Polaris Group to treat can...,Polaris,Group,Files,New,IND,for,ADI-PEG,20,in,Leukemia,medicine,advanced medical technology,medical laboratory technology,medical device technology,latest medical technology,Health
For example, the fact that methylation-dependent silencing of argininosuccinate synthetase (ASS1) [40], a rate-limiting enzyme involved in the biosynthesis of arginine, has been implicated in therapeutic resistance in several cancer types including renal cell carcinoma, hepatocellular carcinoma, malignant melanoma, glioblastoma multiforme, and platinum-resistant epithelial. ovarian cancer suggests a role for demethylating agents in these ASS1 drug-resistant find more cancers [41]. Nevertheless, despite their current nonspecific promiscuity, epigenetic agents may act on most or all tumor types, since aberrant methylation and deacetylation patterns are a hallmark of cancer cells. In particular, several of the anticancer agents described in this review activate and upregulate p53, which itself affects multiple targets [19]. Following genotoxic stress. in response to traditional therapeutic strategies such as cisplatin, doxorubicin, 5-fluorouracil, fludarabine, mitoxantrone, etoposide, or X-ray ...
SWISS-MODEL Template Library (SMTL) entry for 1kh3.1. Crystal Structure of Thermus thermophilus HB8 Argininosuccinate Synthetase in complex with inhibitor
Immune cells in the brain may be abnormally consuming the nutrient arginine, and this may be a cause of Alzheimers disease: new study.
Principal Investigator:MORI Masataka, Project Period (FY):1998 - 2000, Research Category:Grant-in-Aid for Scientific Research (B)., Section:展開研究, Research Field:Pathological medical chemistry
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Homework: The Punnet squares show the enzymatic [colour] and growth [+ or -] phenotypes of two crosses between two dihybrid auxotrophic mutants Assume dominance of the wild-type allele at each locus (x+). What general principles can be drawn from the patterns, as related to tri-hybrid crosses, and other forms of gene interactions in metabolic pathways ...
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Reviews for Wonderfalls - Season 1, Episode 8: Lovesick Ass: As Jaye and Eric verbally spar over their relationship, or more precisely, lack of one, they encounter and help a ...
Reviews for Wonderfalls - Season 1, Episode 8: Lovesick Ass: As Jaye and Eric verbally spar over their relationship, or more precisely, lack of one, they encounter and help a ...
Nivelurile mai scăzute ale vitaminei D au fost asociate cu o prevalență mai mare a rinitei alergice numai la copii. Nivelul mediu al vitaminei D la pacienții cu rinită alergică a fost mai mic decât cel al martorilor doar la copii .
Just because that wasnt dramatic enough, after the surgery, the doctor decided to verbally attack us as a group. He felt like we were questioning him too much, said that if we had some issue with him that we had better find us another doctor, that we had better watch out, and basically threatened to remove himself if we didnt treat him with respect. This was all provoked only by Dads wife asking if she needed to be in on a meeting between doctors (she was joking, and laughed when she said it). Obviously we are all under stress, but this took us completely off guard. We all felt it best to just apologize and kiss his ass, since he is our only hope for my Dad... but talk about knocking us on our asses. We stood around and just stared at each other for a minute...Did that just happen ...
This is not an admission that I think this thing will sell gangbusters, but its a good idea...why? You hit the $99 price point and auto-magically you...
First ah Emmah comes.....Then ah I comes .....then ah 2 asses come..then I ah comes again ...then then ah two asses come again.... then ah I comes again...then then ah Pee 2 times..... and then I comes one final time ...
It wasnt pleasant. The nasty bit of the procedure took about 10 mins or more - hard to say ... but the bit where they pull out the blood and bone ... oh god, I dont want to write about it anymore because its making me squeemish again. All you really need to know was that I ended up calling the doctor a f**king b*stard, which I later apologied for ...
Ultrasonikatioun an Sonochemie, dat ass dApplikatioun vun héijer Kraaft Ultraschall op chemesch Systemer, gi wäit benotzt fir qualitativ héichwäerteg Nano-Gréisst Materialien ze produzéieren (zB Nanopartikelen, Nano-Emulsiounen). Sonikatioun an Sonochemie erméiglechen dProduktioun vun héich performante Nano-Gréisst Materialien oder erliichtert se. De Virdeel vun der Ultraschall-Synthese vun Nano-Partikelen ass dEinfachheet an dEffektivitéit. Wärend alternativ Produktiounsmethoden vun nano-strukturéierte Materialien héich Bulk-Temperaturen, Drock an / oder laang Reaktiounszäiten erfuerderen, erlaabt dUltraschall-Synthese dacks eng einfach, séier an effizient Produktioun vun Nanomaterialien. Béid, sonochemesch a sonomechanesch Effekter generéiert duerch Ultraschall mat héijer Intensitéit si verantwortlech fir dSynthese oder dFunktionaliséierung / Modifikatioun vun Nano-Gréisst Partikelen. Kupplung vun héijer Kraaft Ultraschallwellen a Flëssegkeeten ergëtt akustesch ...
http://www.sobadass.me/wp-content/uploads/2015/09/sobadasslogo.png 0 0 Timm http://www.sobadass.me/wp-content/uploads/2015/09/sobadasslogo.png Timm2017-02-16 09:06:182017-02-16 09:10:11img_9762 ...
This time I wrote to you a video in which I fill my panties plenty of shit , and sit on a chair and begin ass to push shit that is in my shorts . The result of all my white panties and ass shit .. (207). ...
First ah Emmah comes.....Then ah I comes .....then ah 2 asses come..then I ah comes again ...then then ah two asses come again.... then ah I comes again...then then ah Pee 2 times..... and then I comes one final time ...
Za dobivanje zdravih proizvoda u industrijskoj proizvodnji mesnih emulzija važno je smanjiti udjel fosfata. Ispitan je utjecaj sedam vrsta fosfata na fizikalno-kemijska svojstva te kakvoću proizvoda radi odabira najoptimalnijeg spoja...
Za dobivanje zdravih proizvoda u industrijskoj proizvodnji mesnih emulzija važno je smanjiti udjel fosfata. Ispitan je utjecaj sedam vrsta fosfata na fizikalno-kemijska svojstva te kakvoću proizvoda radi odabira najoptimalnijeg spoja...
v Afgn s Al-Kaidou nechteji mit nic moc spolecneho, nemaji radi araby. Usama je vice pod protektoratem pakistanskych warlordu nez Talibancu samotnych i kdyz jiste vazby zde jsou, hlavne financni. Prostrednictvim Usamy vudci Talibanu dostavaji penize na zbrane. Usama bi9n Laden je celkem dost bezvyznamna figurka co se terorismu tyce jsou jeho akce velmi limitovane, Usamove spojeni s 9/11 nebylo nikdy prokazano a jeho paska kde to mel rici, nikdy nebyla stvrzena pravost. Taliban ma v Afgn respekt a zastituje momentalne osvobozenecke hnuti mudzhedinu. Jak rusove museli odejit odejdou i americani .... s neuspechem a haldou mrtvych za nimi. ...
Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. *Carbamoyl phosphate synthetase ...
Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. *Carbamoyl phosphate synthetase ... This entry also includes folylpolyglutamate synthase that transfers glutamate to folylpolyglutamate and cyanophycin synthetase ...
Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. *Carbamoyl phosphate synthetase ... Carbamoyl phosphate synthase (CPSase) is a heterodimeric enzyme composed of a small and a large subunit (with the exception of ... Carbamoyl phosphate synthase has three main steps in its mechanism and is, in essence, irreversible.[4] ... "Inhibition of carbamoyl-phosphate synthase (ammonia) by Tris and Hepes. Effect on Ka for N-acetylglutamate" (PDF). Biochem. J ...
Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. *Carbamoyl phosphate synthetase ... In enzymology, a phosphoribosylformylglycinamidine synthase (EC 6.3.5.3) is an enzyme that catalyzes the chemical reaction ... Retrieved from "https://en.wikipedia.org/w/index.php?title=Phosphoribosylformylglycinamidine_synthase&oldid=808965560" ...
Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. *Carbamoyl phosphate synthetase ...
Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. *Carbamoyl phosphate synthetase ... Asparagine synthase (glutamine-hydrolysing) (EC 6.3.5.4, asparagine synthetase (glutamine-hydrolysing), glutamine-dependent ... Retrieved from "https://en.wikipedia.org/w/index.php?title=Asparagine_synthase_(glutamine-hydrolysing)&oldid=917335771" ...
Argininosuccinate synthase. *Argonaute. *Argos (EGFR Inhibitor). *Aromatic L-amino acid decarboxylase. *Arrestin ...
Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. *Carbamoyl phosphate synthetase ... "Synthases and ligases". chem.qmul.ac.uk. Archived from the original on October 15, 2012. Retrieved July 28, 2013.. .mw-parser- ... Under one definition, synthases do not use energy from nucleoside triphosphates (such as ATP, GTP, CTP, TTP, and UTP), whereas ... It is also said that a synthase is a lyase (a lyase is an enzyme that catalyzes the breaking of various chemical bonds by means ...
The enzyme CTP synthase catalyzes the next reaction step: the conversion of UTP to CTP by transferring an amino group from ... Then, the enzymes citrulline and argininosuccinate convert ornithine to arginine. There are two distinct lysine biosynthetic ... 4-hydroxy-tetrahydrodipicolinate synthase adds a pyruvate group to the β-aspartyl-4-semialdehyde, and a water molecule is ... The next reaction is catalyzed by the enzyme pyrroline-5-carboxylate synthase (P5CS), which catalyzes the reduction of the ϒ- ...
In humans, argininosuccinate lyase (ASL) is a homotetrameric enzyme that can undergo intragenic complementation. An ASL ... "Structure of a Heterotetrameric Geranyl Pyrophosphate Synthase from Mint (Mentha piperita) Reveals Intersubunit Regulation". ... Turner MA, Simpson A, McInnes RR, Howell PL (August 1997). "Human argininosuccinate lyase: a structural basis for intragenic ... Yu B, Howell PL (October 2000). "Intragenic complementation and the structure and function of argininosuccinate lyase". Cell. ...
"Gene sharing by delta-crystallin and argininosuccinate lyase". Proceedings of the National Academy of Sciences of the United ... "Crystal structure of albaflavenone monooxygenase containing a moonlighting terpene synthase active site". The Journal of ...
N-acetylneuraminate synthase NINJ1: ninjurin-1 NOL6: nucleolar protein 6 NUDT2: nudix hydrolase 2 OLFM1: olfactomedin 1 PHF2: ... argininosuccinate synthetase BNC2: zinc finger protein basonuclin-2 C9orf64: chromosome 9 open reading frame 64 C9orf78: ...
Argininosuccinate synthetase (EC 6.3.4.5). *CTP synthase (EC 6.3.4.2). Category:EC 6.4 (form carbon-carbon bonds)Edit. * ... EC 2.9.1.2: O-phospho-L-seryl-tRNA(Sec):L-selenocysteinyl-tRNA synthase ... 2-Succinyl-5-enolpyruvyl-6-hydroxy-3-cyclohexene-1-carboxylic-acid synthase EC 2.2.1.9 ... EC 6.2.1.38: (2,2,3-trimethyl-5-oxocyclopent-3-enyl)acetyl-CoA synthase ...
This reaction is ATP dependent and is catalyzed by argininosuccinate synthetase. Argininosuccinate undergoes cleavage by ... Synthesis of NAcGlu by N-acetylglutamate synthase (NAGS) is stimulated by both Arg, allosteric stimulator of NAGS, and Glu, a ... The reactions of the urea cycle 1 L-ornithine 2 carbamoyl phosphate 3 L-citrulline 4 argininosuccinate 5 fumarate 6 L-arginine ... N-Acetylglutamate synthase (NAGS) deficiency Carbamoyl phosphate synthetase (CPS) deficiency Ornithine transcarbamoylase (OTC) ...
... and N-Acetylglutamate synthase deficiency. Other diseases that may appear similar to CTLN1 include the organic acidemias and ... is a rare disease caused by a deficiency in argininosuccinate synthetase, an enzyme involved in excreting excess nitrogen from ...
Argininosuccinate synthase or synthetase (ASS; EC 6.3.4.5) is an enzyme that catalyzes the synthesis of argininosuccinate from ... In humans, argininosuccinate synthase is encoded by the ASS gene located on chromosome 9. ASS is responsible for the third step ... Haines RJ, Pendleton LC, Eichler DC (2011). "Argininosuccinate synthase: at the center of arginine metabolism". International ... The enzyme endothelial nitric oxide synthase produces nitric oxide from arginine in endothelial cells. Argininosuccinate ...
Argininosuccinate synthase (EC:6.3.4.5) (AS) is a urea cycle enzyme that catalyzes the penultimate step in arginine ... Argininosuccinate synthase, type 2 subfamily (IPR023437). Short name: Arg_succ_synth_type2_subfam ... Conservation of structure in the human gene encoding argininosuccinate synthetase and the argG genes of the archaebacteria ... Sequences of the genes encoding argininosuccinate synthetase in Escherichia coli and Saccharomyces cerevisiae: comparison with ...
Argininosuccinate synthase (ASS1), Argininosuccinate synthase (ASS1), Citrulline--aspartate ligase (CN), Citrulline--aspartate ... Argininosuccinate synthase (ASS1), Argininosuccinate synthase (ASS1), Citrulline--aspartate ligase (CN), Citrulline--aspartate ... Argininosuccinate synthaseAdd BLAST. 412. Amino acid modifications. Feature key. Position(s). DescriptionActions. Graphical ... Argininosuccinate synthaseCurated (EC:6.3.4.5*Search proteins in UniProtKB for this EC number. ...
Belongs to the argininosuccinate synthase family. Type 2 subfamily.UniRule annotation. Manual assertion according to rulesi ... sp,A0AYH4,ASSY_BURCH Argininosuccinate synthase OS=Burkholderia cenocepacia (strain HI2424) OX=331272 GN=argG PE=3 SV=1 ... Argininosuccinate lyase (argH). This subpathway is part of the pathway L-arginine biosynthesis, which is itself part of Amino- ... 2-(Nω-L-arginino)succinate*Search proteins in UniProtKB for this molecule. ...
The worlds first wiki where authorship really matters. Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts.
ASS, CTLN1, EC 6.3.4.5, ASS1, Argininosuccinate Synthase 1.. Introduction. ASS1 is involved in the urea cycle, which is a ...
Argininosuccinate synthase 1 is an intrinsic Akt repressor transactivated by p53. By Takafumi Miyamoto, Paulisally Hau Yi Lo, ... Argininosuccinate synthase 1 is an intrinsic Akt repressor transactivated by p53. By Takafumi Miyamoto, Paulisally Hau Yi Lo, ... Argininosuccinate synthase 1 is an intrinsic Akt repressor transactivated by p53 Message Subject. (Your Name) has forwarded a ... We show that argininosuccinate synthase 1 (ASS1), a citrulline-aspartate ligase in de novo arginine synthesis pathway, was ...
Various tumor types have been shown to lack expression of the enzyme argininosuccinate synthase (ASS) that is required for the ... Abstract 4519: Small cell lung cancers lack expression of argininosuccinate synthase (ASS) and are sensitive to arginine ... Abstract 4519: Small cell lung cancers lack expression of argininosuccinate synthase (ASS) and are sensitive to arginine ... Abstract 4519: Small cell lung cancers lack expression of argininosuccinate synthase (ASS) and are sensitive to arginine ...
ASS1 argininosuccinate synthase 1 [ Homo sapiens ]. Synonyms:. ASS1; argininosuccinate synthase 1; ASS; CTLN1; ... Recombinant Human Argininosuccinate Synthase 1, His-tagged. Download Datasheet See All ASS1 Products. Bring this labeled ... The ASS1 is an enzyme called argininosuccinate synthase 1. This enzyme participates in the urea cycle, which is a sequence of ... argininosuccinate synthase; OTTHUMP00000022363; OTTHUMP00000022364; citrulline-aspartate ligase; citrulline--aspartate ligase; ...
argininosuccinate synthase. CPA. N-carbamoylputrescine amidohydrolase. CPS. carbamoylsynthetase. dSAM. decarboxylated S- ...
ASS1: argininosuccinate synthase 1. *ASXL1: ASXL transcriptional regulator 1. *ATG16L1: autophagy related 16 like 1 ...
The ASS1 gene provides instructions for making an enzyme called argininosuccinate synthase 1. This enzyme participates in the ... Argininosuccinate synthase 1 is involved in the third step of the urea cycle. This step combines two protein building blocks ( ... Defects in argininosuccinate synthase 1 disrupt the third step of the urea cycle, preventing the liver from processing excess ... The ASS1 gene provides instructions for making an enzyme called argininosuccinate synthase 1. This enzyme participates in the ...
Argininosuccinate synthase. MTTILKHLPVGQRIGIAFSGGLDTSAALLWMRQKGAVPYAYTANLGQPDE.... unknown. Enolase. ... Hematopoietic prostaglandin D synthase. MPNYKLTYFNMRGRAEIIRYIFAYLDIQYEDHRIEQADWPEIKSTLPFGK.... unknown. Endoglucanase G. ... Inositol-3-phosphate synthase 1. MEAAAQFFVESPDVVYGPEAIEAQYEYRTTRVSREGGVLKVHPTSTRFTF.... unknown. C4-dicarboxylate transport ...
ASS1 deficiency -- Argininosuccinate synthase 1. *Citrin deficiency -- Citrin. *CPSI deficiency -- Carbamoyl phosphate ...
Argininosuccinate synthase (IPR001518) Pfam signature: PF00764 Autoinducer synthase (IPR001690) Pfam signature: PF00765 ... GMP synthase, C-terminal (IPR001674) Pfam signature: PF00958 Glycoside hydrolase, family 24 (IPR002196) Pfam signature: PF00959 ... Pseudouridine synthase I, TruA, alpha/beta domain (IPR020097) Pfam signature: PF01416 ENTH domain (IPR013809) Pfam signature: ... Thymidylate synthase/dCMP hydroxymethylase domain (IPR023451) Pfam signature: PF00303 Unintegrated signature Pfam signature: ...
Argininosuccinate synthase; RCC1: Regulator of chromosome condensation; MFGE8: Lactadherin; FSCN1: Fascin; LDHB: L-lactate ...
Abnormal gene product. The argininosuccinate synthase enzyme is inactive or absent. Mutated ASS with abnormal KM (Michaelis ... Argininosuccinate synthase enzyme activity can be measured in uncultured fetal tissue obtained by chorionic villus sampling ( ... Normal gene product. The translational product, argininosuccinate synthase, is a homotetramer of 186 kd. It catalyzes an ... Argininosuccinate synthase (ASS) enzyme activity. Incorporation of radiolabeled citrulline into argininosuccinic acid is ...
The up-regulation of argininosuccinate synthase participated in proline biosynthesis at 16% MC, which is important for ... The down-regulated proteins, notably six heat shock proteins and two ATP synthases, have important roles in the mobilization of ... including ATP synthase subunit alpha, argininosuccinate synthase (ASS), and 17.3, 17.9, and 18.3 kDa class I heat shock ... The up-regulation of argininosuccinate synthase participated in proline biosynthesis at 16% MC, which is important for ...
AS, argininosuccinate synthase; CINC, cytokine-induced neutrophyl chemoattractant; CKK, cholecystokinin; COX, cyclooxygenase; ... argininosuccinate synthase (AS) (increased), ornithine decarboxilase (increased), hsp-70 (increased), MnSOD (increased), HO-1 ( ... Eizirik DL, Flodström M, Karlsen AE, Welsh N: The harmony of the spheres: inducible nitric oxide synthase and related genes in ... Corbett JA, Kwon G, Turk J, McDaniel ML: IL-1β induces the coexpression of both nitric oxide synthase and cyclooxygenase by ...
CPS, carbamoyl phosphate synthase; OTC, ornithine carbamoyltransferase; AsuS, argininosuccinate synthase; AsL, ... As for arginine, it is used by nitric oxide synthase to synthesize nitric oxide, an important signaling molecule in plants and ... line having a reduced level of carbamoyl phosphate synthase, the enzyme catalyzing the first committed step of the OUC (Allen ... argininosuccinate lyase; Arg*, arginase (depicted twice, because it produces both ornithine and urea); Ure, urease. ...
Argininosuccinate synthase: at the center of arginine metabolism. Int J Biochem Mol Biol. 2011;2(1):8-23.PubMedGoogle Scholar ... Argininosuccinate synthetase overexpression in vascular smooth muscle cells potentiates immunostimulant-induced NO production. ... Arginine itself is a known vasodilator and CM indirectly increases vasodilation and production of nitrogen oxygen synthase (NOS ...
Belongs to the argininosuccinate synthase family. Type 1 subfamily.. * Target information above from: UniProt accession P00966 ... Nitrogen metabolism; urea cycle; (N(omega)-L-arginino)succinate from L-aspartate and L-citrulline: step 1/1. ...
YHR018C ARG4; argininosuccinate lyase ARG4 YOL058W ARG1; argininosuccinate synthase YMR054W STV1; H(+)-transporting V0 sector ... argininosuccinate lyase [EC:4.3.2.1] K01940 argG; argininosuccinate synthase [EC:6.3.4.5] K02154 ATPeV0A; V-type H+- ... YOL049W GSH2; glutathione synthase YGL040C HEM2; porphobilinogen synthase HEM2 YGR244C LSC2; succinate--CoA ligase (GDP-forming ... glutathione synthase [EC:6.3.2.3] K01698 hemB; porphobilinogen synthase [EC:4.2.1.24] K01900 LSC2; succinyl-CoA synthetase beta ...
2014). Molecular characterization of argininosuccinate synthase and argininosuccinate lyase from the liver of the African ... argininosuccinate synthase, Chng et al., 2014) and others that have greater similarity to those of tetrapods (e.g. F2 and Fgg, ... this study; argininosuccinate lyase, Chng et al., 2014; Na+/K+-ATPase α-subunit isoforms, Hiong et al., 2014; l-gulono-γ- ...
ASL, argininosuccinate lyase; Asp, aspartate; ASS, argininosuccinate synthase; BH4, tetrahydrobiopterin; DCAM, decarboxylated 5 ... 1999) Effects of the new arginase inhibitor Nω-hydroxy-nor-l-arginine on NO synthase activity in murine macrophages. Nitric ... Almost all mammalian cells contain one of the isoforms of nitric oxide (NO) synthase (NOS) that synthesizes NO and l-citrulline ... 1997) Coinduction of nitric oxide synthase and arginase I in cultured rat peritoneal macrophages and rat tissues in vivo by ...
CS053_06215 argH; argininosuccinate lyase CS053_06190 argininosuccinate synthase CS053_03570 PLP-dependent transferase CS053_ ... argininosuccinate lyase [EC:4.3.2.1] K01940 argG; argininosuccinate synthase [EC:6.3.4.5] K01758 CTH; cystathionine gamma-lyase ... bifunctional tetrahydrofolate synthase/dihydrofolate synthase CS053_14485 ribB; 3,4-dihydroxy-2-butanone-4-phosphate synthase ... CS053_04265 queE; 7-carboxy-7-deazaguanine synthase QueE CS053_04270 queC; 7-cyano-7-deazaguanine synthase QueC CS053_17595 ...
ARG, arginase; ASS, argininosuccinate synthase; ASL, argininosuccinate lyase; CPS1, carbamoylphosphate I synthase; ORNT 1, ... Argininosuccinate aciduria, citrullinemia type I homocystinuria (cystathionine-β-synthase), maple syrup urine disease, ... CBS, cystathionine β synthase; Cbl, cobalamin; SAM, S-adenosyl methionine; SAH, S-adenosylhomocysteine; MAT, methionine ... The classic homocystinuria is caused by cystathionine β-synthase (CBS) deficiency [10], a key enzyme in the trans-sulfuration ...
Argininosuccinate Lyase / analysis. Argininosuccinate Synthase / analysis. Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing) ... EC 4.3.2.1/Argininosuccinate Lyase; EC 6.3.4.5/Argininosuccinate Synthase; EC 6.3.5.5/Carbamoyl-Phosphate Synthase (Glutamine- ... did not induce any of the urea cycle enzymes in adrenalectomized Sprague-Dawley rats and only induced argininosuccinate lyase ( ...
ASS1: argininosuccinate synthase 1. *ASXL1: ASXL transcriptional regulator 1. *ATG16L1: autophagy related 16 like 1 ...
UArgininosuccinate synthase. Not Available. Escherichia coli (strain K12). UGuanidinoacetate N-methyltransferase. Not Available ...
  • We show that argininosuccinate synthase 1 ( ASS1 ), a citrulline-aspartate ligase in de novo arginine synthesis pathway, was directly transactivated by p53 in response to genotoxic stress, resulting in the rearrangement of arginine metabolism. (sciencemag.org)
  • The ASS1 is an enzyme called argininosuccinate synthase 1. (creativebiomart.net)
  • The ASS1 gene provides instructions for making an enzyme called argininosuccinate synthase 1. (medlineplus.gov)
  • Diez-Fernandez C, Rüfenacht V, Häberle J. Mutations in the Human Argininosuccinate Synthetase (ASS1) Gene, Impact on Patients, Common Changes, and Structural Considerations. (medlineplus.gov)
  • An important gene associated with Citrullinemia, Classic is ASS1 (Argininosuccinate Synthase 1), and among its related pathways/superpathways are Arginine biosynthesis and Alanine, aspartate and glutamate metabolism . (malacards.org)
  • The team of scientists examined two types citrullinaemia ( amino acid disorders that do not allow cells to produce citrin--which is essential to removing urea, ammonia, and other toxic substances from the blood via the liver) to show that levels of Argininosuccinate synthase (ASS1), the missing enzyme that. (umd.edu)
  • Citrullinemia type I is an autosomal recessive disorder that is caused by a deficiency of the urea cycle enzyme argininosuccinate synthetase (ASS1). (ptglab.com)
  • Yuan, Yang;Mohammad, Mahmoud A;Betancourt, Ancizar;Didelija, Inka C;Yallampalli, Chandrasekar;Marini, Juan C 2018-06-07 00:00:00 Abstract Background The recycling of citrulline by argininosuccinate synthase 1 (ASS1) and argininosuccinate lyase (ASL) is crucial to maintain arginine availability and nitric oxide (NO) production. (deepdyve.com)
  • The availability of arginine seems to limit the synthesis of NO, because local arginase activity (5) or arginine supplementation (6) affects the rate of production of NO. Citrulline has the potential to be recycled intracellularly through the action of argininosuccinate synthase 1 (ASS1) and argininosuccinate lyase (ASL) to regenerate a new arginine molecule and thus sustain NO production (7, 8). (deepdyve.com)
  • But a multi-center phase 2 randomized clinical trial demonstrated that 68 of 201 mesothelioma tumors were deficient in argininosuccinate synthase (ASS1) , an enzyme necessary for arginine synthesis. (survivingmesothelioma.com)
  • Glucagon did not induce any of the urea cycle enzymes in adrenalectomized Sprague-Dawley rats and only induced argininosuccinate lyase (EC 4.3.2.1) in adrenalectomized inbred Wistar-Furth rats. (biomedsearch.com)
  • Arginine is synthesized from citrulline through two-step reactions catalyzed by AS and argininosuccinate lyase, in which AS is the rate-limiting enzyme. (aacrjournals.org)
  • Braissant O, Honegger P, Loup M, Iwase K, Takiguchi M, Bachmann C: Hyperammonemia: regulation of argininosuccinate synthetase and argininosuccinate lyase genes in aggregating cell cultures of fetal rat brain. (hmdb.ca)
  • In support of this hypothesis, the recycling enzymes, argininosuccinate synthase (AS) and argininosuccinate lyase (AL), have been shown to colocalize with eNOS in caveolae, a subcompartment of the plasma membrane. (biologists.org)
  • The synergistic effect was independent of basic and induced argininosuccinate synthase or argininosuccinate lyase protein expression and not abrogated by the presence of citrulline. (aacrjournals.org)
  • The kidney is the major organ for circulating L-Arg synthesis, utilizing citrulline conversion by argininosuccinate synthase (ASS) and lyase (ASL). (ufl.edu)
  • In the cytoplasm, L-ornithine is converted to L-arginine in three reactions mediated by ornithine carbamoyltransferase, arginosuccinate synthase, and argininosuccinate lyase. (wikipathways.org)
  • It is also said that a synthase is a lyase (a lyase is an enzyme that catalyzes the breaking of various chemical bonds by means other than hydrolysis and oxidation, often forming a new double bond or a new ring structure) and does not require any energy, whereas a synthetase is a ligase (a ligase is an enzyme that binds two chemicals or compounds) and thus requires energy. (wikipedia.org)
  • Zhang B, Cao GL, Domachowske J, Jackson MJ, Porasuphatana S, Rosen GM: Stable expression of varied levels of inducible nitric oxide synthase in primary cultures of endothelial cells. (hmdb.ca)
  • Zhang WY, Gotoh T, Oyadomari S, Mori M: Coinduction of inducible nitric oxide synthase and arginine recycling enzymes in cytokine-stimulated PC12 cells and high output production of nitric oxide. (hmdb.ca)
  • The enzyme endothelial nitric oxide synthase (eNOS) catalyzes the conversion of arginine, oxygen and NADPH to NO and citrulline. (biologists.org)
  • Endothelial nitric oxide synthase (eNOS), the enzyme that catalyzes the production of NO from the amino acid arginine in endothelial cells, plays a key role in vasoregulation as well as in other important physiological processes such as angiogenesis. (biologists.org)
  • Repeated induction of nitric oxide synthase and leishmanicidal activity in murine macrophages. (semanticscholar.org)
  • Murine macrophages express high levels of nitric oxide (NO) synthase and produce large amounts of NO when stimulated with interferon-gamma plus lipopolysaccharide in vitro. (semanticscholar.org)
  • The abnormal regulation of inducible nitric oxide synthase (iNOS) and neuronal nitric oxide synthase (nNOS) is associated with neurodegenerative disorders. (hindawi.com)
  • Nitric oxide (NO) is important for many synthases (NOSs, EC 1.14.13.39), including inducible nitric oxide synthase (iNOS), neuronal nitric oxide synthase (nNOS), and endothelial nitric oxide synthase (eNOS) [ 1 ]. (hindawi.com)
  • NOS, nitric oxide synthase. (deepdyve.com)
  • This radiosensitization of the tumor cells was inhibited by a nitric oxide (NO) synthase inhibitor so they realized that this sensitization was due to NO stimulation. (denvernaturopathic.com)
  • Nitric oxide recycling is an important function that can sometimes be inadequate in those with the B antigen (blood groups B and AB), probably due to genetic linkage of the argininosuccinate synthase enzyme. (dadamo.com)
  • Biochemical nomenclature has sometimes distinguished synthetases from synthases and sometimes treated the words as synonyms. (wikipedia.org)
  • Increased metabolism of l -Arginine ( l -Arg), through the enzymes arginase 1 and NO synthase 2 (NOS2), is well documented as a major MDSC suppressive mechanism. (jimmunol.org)
  • Pharmaceutical grade L-citrulline supplements are used for carbamoylphosphate synthetase (CPS) and ornithine transcarbamylase (OTC) deficiency and L-arginine is used in case of argininosuccinate synthase deficiency and citrullinemia to catalyze the enzymes in urea cycle and support optimum ammonia removal. (news-medical.net)
  • Importantly, lack of ASS also influences essential enzymes in pyrimidine synthesis (carbamoyl-phosphate synthetase2, aspartate transcarbamylase and dihydrooratase (CAD) and thymidylate synthase (TS)) and malate dehydrogenase-1 (MDH-1) in TCA cycle. (elsevier.com)
  • Increased expression and activities of arginase 1 (ARG1) and NO synthase 2 (NOS2) are well established as the hallmarks for MDSC suppressive function ( 11 ). (jimmunol.org)
  • Sequences of the genes encoding argininosuccinate synthetase in Escherichia coli and Saccharomyces cerevisiae: comparison with methanogenic archaebacteria and mammals. (ebi.ac.uk)
  • Conservation of structure in the human gene encoding argininosuccinate synthetase and the argG genes of the archaebacteria Methanosarcina barkeri MS and Methanococcus vannielii. (ebi.ac.uk)
  • Argininosuccinate Synthase 1-Deficiency Enhances the Cell Sensitivity to Arginine through Decreased DEPTOR Expression in Endometrial Cancer. (abcam.com)
  • We studied the possible mechanisms of NO deficiency in various models of CKD, focusing on possible reduced NO synthase (NOS) substrate (L-arginine, L-Arg). (ufl.edu)
  • Most of the mutations involved in type I citrullinemia change single amino acids in the argininosuccinate synthase 1 enzyme. (medlineplus.gov)
  • Structure of the human argininosuccinate synthetase gene and an improved system for molecular diagnostics in patients with classical and mild citrullinemia. (medlineplus.gov)
  • In the context of a proteomic screening of intestinal modifications induced by dietary spermine in suckling rats, we showed that argininosuccinate synthetase and carbamoyl phosphate synthase disappeared from enterocytes after this treatment. (ac.be)
  • Various tumor types have been shown to lack expression of the enzyme argininosuccinate synthase (ASS) that is required for the synthesis of arginine and thus rely on exogenous sources for protein synthesis requirements. (aacrjournals.org)
  • Four proteins, namely, heat shock 70 kDa protein 1, argininosuccinate synthase, isoform 2 of UTP-glucose-1-phosphate uridylyltransferase, and transketolase, were shown to have the potential to differentiate metastatic relapse (MR) from nonrelapse (NR) HCC patients after validation by western blotting and immunohistochemical assays. (sigmaaldrich.com)
  • Argininosuccinate synthase ( EC:6.3.4.5 ) (AS) is a urea cycle enzyme that catalyzes the penultimate step in arginine biosynthesis: the ATP-dependent ligation of citrulline to aspartate to form argininosuccinate, AMP and pyrophosphate [ PMID: 2123815 , PMID: 3133361 ]. (ebi.ac.uk)
  • 1.73 Angstrom Resolution Crystal Structure of Dihydropteroate Synthase (folP-SMZ_B27) from Soil Uncultured Bacterium. (csgid.org)
  • This entry represents argininosuccinate synthase, type 2 subfamily. (ebi.ac.uk)
  • The down-regulated proteins, notably six heat shock proteins and two ATP synthases, have important roles in the mobilization of carbohydrates and energy, and in the balance between synthesis and degradation of other proteins during seed deterioration. (frontiersin.org)
  • ADI-PEG20, arginine, argininosuccinate, citrulline, mouse, recycling Introduction Arginine is a conditionally indispensable amino acid that serves as a precursor for the synthesis of numerous compounds. (deepdyve.com)
  • The synthesis of NO is catalyzed by the oxidation of the imino group of arginine by intracellular NO synthase (NOS), generating citrulline as a coproduct of this reaction (4) (Figure 1). (deepdyve.com)
  • However, the Joint Commission on Biochemical Nomenclature (JCBN) dictates that "synthase" can be used with any enzyme that catalyses synthesis (whether or not it uses nucleoside triphosphates), whereas "synthetase" is to be used synonymously. (wikipedia.org)
  • Loss of argininosuccinate synthetase (ASS) expression in melanoma makes these tumor cells vulnerable to arginine deprivation. (elsevier.com)
  • The up-regulation of argininosuccinate synthase participated in proline biosynthesis at 16% MC, which is important for maintaining reactive oxygen species homeostasis for the resistance of heat stress. (frontiersin.org)
  • The sensitivity of melanoma to ADI treatment is based on its auxotrophy for arginine due to a lack of argininosuccinate synthetase (AS) expression, the rate-limiting enzyme for the de novo biosynthesis of arginine. (aacrjournals.org)
  • Tresch S, Heilmann M, Christiansen N, Looser R, Grossmann K. Inhibition of saturated very-long-chain fatty acid biosynthesis by mefluidide and perfluidone, selective inhibitors of 3-ketoacyl-CoA synthases. (labome.org)
  • Argininosuccinate synthetase (ASS), a key enzyme to synthesize arginine is down regulated in many tumors including hepatocellular carcinoma (HCC). (elsevier.com)
  • Although the importance of both intestinal and renal argininosuccinate synthetases has been recognised for a long time, nutrients have not yet been identified as inducers of the gene expression. (ac.be)
  • Argentilactone inhibited the enzymatic activity of malate dehydrogenase, citrate synthase, and pyruvate dehydrogenase. (asm.org)
  • Defects in argininosuccinate synthase 1 disrupt the third step of the urea cycle, preventing the liver from processing excess nitrogen into urea. (medlineplus.gov)
  • Argininosuccinate synthetase, an ubiquitous enzyme in mammals, catalyses the formation of argininosuccinate, the precursor of arginine. (ac.be)
  • Genetic Manipulation of Leishmania donovani to Explore the Involvement of Argininosuccinate Synthase in Oxidative Stress Management. (semanticscholar.org)
  • It has been reported that majority of melanomas and hepatocellular carcinomas are auxotrophic for arginine because of their lack of argininosuccinate synthetase (AS) expression ( 3 - 5 ). (aacrjournals.org)
  • The expression of NO synthase peaks at 12 h after stimulation and declines rapidly to the background level by 72 h. (semanticscholar.org)
  • Following MDI aerosol exposure, expression of a number of proteins with immunological or xenobiotic metabolism relevance is increased, including endoplasmin, cytochrome P450 and argininosuccinate synthase. (cdc.gov)
  • Belongs to the argininosuccinate synthase family. (abcam.com)