An enzyme of the urea cycle which splits argininosuccinate to fumarate plus arginine. Its absence leads to the metabolic disease ARGININOSUCCINIC ACIDURIA in man. EC 4.3.2.1.
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.
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.
This amino acid is formed during the urea cycle from citrulline, aspartate and ATP. This reaction is catalyzed by argininosuccinic acid synthetase.
A class of enzymes that catalyze the cleavage of C-C, C-O, and C-N, and other bonds by other means than by hydrolysis or oxidation. (Enzyme Nomenclature, 1992) EC 4.
A ureahydrolase that catalyzes the hydrolysis of arginine or canavanine to yield L-ornithine (ORNITHINE) and urea. Deficiency of this enzyme causes HYPERARGININEMIA. EC 3.5.3.1.
A heterogeneous family of water-soluble structural proteins found in cells of the vertebrate lens. The presence of these proteins accounts for the transparency of the lens. The family is composed of four major groups, alpha, beta, gamma, and delta, and several minor groups, which are classed on the basis of size, charge, immunological properties, and vertebrate source. Alpha, beta, and delta crystallins occur in avian and reptilian lenses, while alpha, beta, and gamma crystallins occur in all other lenses.
An essential amino acid that is physiologically active in the L-form.
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.
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.
A group of diseases related to a deficiency of the enzyme ARGININOSUCCINATE SYNTHASE which causes an elevation of serum levels of CITRULLINE. In neonates, clinical manifestations include lethargy, hypotonia, and SEIZURES. Milder forms also occur. Childhood and adult forms may present with recurrent episodes of intermittent weakness, lethargy, ATAXIA, behavioral changes, and DYSARTHRIA. (From Menkes, Textbook of Child Neurology, 5th ed, p49)
A urea cycle enzyme that catalyzes the formation of orthophosphate and L-citrulline (CITRULLINE) from CARBAMOYL PHOSPHATE and L-ornithine (ORNITHINE). Deficiency of this enzyme may be transmitted as an X-linked trait. EC 2.1.3.3.
A 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 amino acid produced in the urea cycle by the splitting off of urea from arginine.
A class of enzymes that catalyze the formation of a bond between two substrate molecules, coupled with the hydrolysis of a pyrophosphate bond in ATP or a similar energy donor. (Dorland, 28th ed) EC 6.
A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
An enzyme that, in the presence of ATP and COENZYME A, catalyzes the cleavage of citrate to yield acetyl CoA, oxaloacetate, ADP, and ORTHOPHOSPHATE. This reaction represents an important step in fatty acid biosynthesis. This enzyme was formerly listed as EC 4.1.3.8.
The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.
The insertion of recombinant DNA molecules from prokaryotic and/or eukaryotic sources into a replicating vehicle, such as a plasmid or virus vector, and the introduction of the resultant hybrid molecules into recipient cells without altering the viability of those cells.
The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.
A family of gram-negative aerobic bacteria in the class BETA PROTEOBACTERIA, encompassing the acidovorans rRNA complex. Some species are pathogenic for PLANTS.
A genus of gram-negative, straight or slightly curved rods which are motile by polar flagella and which accumulate poly-beta-hydroxybutyrate within the cells.
A free radical gas produced endogenously by a variety of mammalian cells, synthesized from ARGININE by NITRIC OXIDE SYNTHASE. Nitric oxide is one of the ENDOTHELIUM-DEPENDENT RELAXING FACTORS released by the vascular endothelium and mediates VASODILATION. It also inhibits platelet aggregation, induces disaggregation of aggregated platelets, and inhibits platelet adhesion to the vascular endothelium. Nitric oxide activates cytosolic GUANYLATE CYCLASE and thus elevates intracellular levels of CYCLIC GMP.
A progressive condition usually characterized by combined failure of several organs such as the lungs, liver, kidney, along with some clotting mechanisms, usually postinjury or postoperative.
An NADPH-dependent enzyme that catalyzes the conversion of L-ARGININE and OXYGEN to produce CITRULLINE and NITRIC OXIDE.
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)
A genus of phototrophic, obligately anaerobic bacteria in the family Chlorobiaceae. They are found in hydrogen sulfide-containing mud and water environments.
A phylum of anoxygenic, phototrophic bacteria including the family Chlorobiaceae. They occur in aquatic sediments, sulfur springs, and hot springs and utilize reduced sulfur compounds instead of oxygen.
Pyrrole containing pigments found in photosynthetic bacteria.
Diabetes mellitus induced experimentally by administration of various diabetogenic agents or by PANCREATECTOMY.
KIDNEY injuries associated with diabetes mellitus and affecting KIDNEY GLOMERULUS; ARTERIOLES; KIDNEY TUBULES; and the interstitium. Clinical signs include persistent PROTEINURIA, from microalbuminuria progressing to ALBUMINURIA of greater than 300 mg/24 h, leading to reduced GLOMERULAR FILTRATION RATE and END-STAGE RENAL DISEASE.
An antibiotic that is produced by Stretomyces achromogenes. It is used as an antineoplastic agent and to induce diabetes in experimental animals.
The determination of the pattern of genes expressed at the level of GENETIC TRANSCRIPTION, under specific circumstances or in a specific cell.
A large collection of DNA fragments cloned (CLONING, MOLECULAR) from a given organism, tissue, organ, or cell type. It may contain complete genomic sequences (GENOMIC LIBRARY) or complementary DNA sequences, the latter being formed from messenger RNA and lacking intron sequences.
Widely used technique which exploits the ability of complementary sequences in single-stranded DNAs or RNAs to pair with each other to form a double helix. Hybridization can take place between two complimentary DNA sequences, between a single-stranded DNA and a complementary RNA, or between two RNA sequences. The technique is used to detect and isolate specific sequences, measure homology, or define other characteristics of one or both strands. (Kendrew, Encyclopedia of Molecular Biology, 1994, p503)
The pattern of GENE EXPRESSION at the level of genetic transcription in a specific organism or under specific circumstances in specific cells.
Magnesium oxide (MgO). An inorganic compound that occurs in nature as the mineral periclase. In aqueous media combines quickly with water to form magnesium hydroxide. It is used as an antacid and mild laxative and has many nonmedicinal uses.
A primary malignant neoplasm of epithelial liver cells. It ranges from a well-differentiated tumor with EPITHELIAL CELLS indistinguishable from normal HEPATOCYTES to a poorly differentiated neoplasm. The cells may be uniform or markedly pleomorphic, or form GIANT CELLS. Several classification schemes have been suggested.
Tumors or cancer of the LIVER.

Glucocorticoids mediate the enhanced expression of intestinal type II arginase and argininosuccinate lyase in postweaning pigs. (1/123)

Arginine metabolism is enhanced in the small intestine of weanling pigs, but the molecular mechanism(s) involved is not known. The objectives of this study were to determine the following: 1) whether glucocorticoids play a role in induction of intestinal arginine metabolic enzymes during weaning; 2) whether the induction of enzyme activities was due to increases in corresponding mRNA levels; and 3) the identity of the arginase isoform(s) expressed in the small intestine. Jejunum was obtained from 29-d-old weaned pigs that were or were not treated with 17-beta-hydroxy-11beta-(4-dimethylaminophenyl)17alpha-(prop- 1-ynyl)es tra-4,9-dien-3-one (RU486, an antagonist of glucocorticoid receptors), or from age-matched suckling pigs. Activities and mRNA levels for type I and type II arginases, argininosuccinate synthase (ASS) and argininosuccinate lyase (ASL) were determined. Activities of arginase, ASL and ASS increased by 635, 56 and 106%, respectively, in weanling pigs, compared with suckling pigs. RU486 treatment attenuated the increase in arginase activity by 74% and completely prevented the ASL induction in weanling pigs, but had no effect on ASS activity. Pig intestine expresses both type I and type II arginases. On the basis of immunoblot analyses, there was no significant difference in levels of intestinal type I arginase among these three groups of pigs, indicating that changes in arginase activity were due only to type II arginase. The mRNA levels for type II arginase and ASL increased by 135 and 198%, respectively, in weanling pigs compared with suckling pigs, and this induction was completely prevented by RU486. In contrast, ASS mRNA levels did not differ between suckling and weanling pigs. These results suggest that intestinal type II arginase, ASS and ASL are regulated differentially at transcriptional and post-translational levels and that glucocorticoids play a major role in the induction of type II arginase and ASL mRNAs in the small intestine of weanling pigs.  (+info)

Competitive inactivation of a double-strand DNA break site involves parallel suppression of meiosis-induced changes in chromatin configuration. (2/123)

In Saccharomyces cerevisiae, DNA double-strand breaks (DSBs) initiate meiotic recombination at open sites in chromatin, which display a meiosis-specific increase in micrococcal nuclease (MNase) sensitivity. The arg4 promoter contains such a DSB site. When arg4 sequences are placed in a pBR322-derived insert at HIS4 (his4 :: arg4 ), the presence of strong DSB sites in pBR322 sequences leads to an almost complete loss of breaks from the insert-borne arg4 promoter region. Most of the MNase-sensitive sites occurred at similar positions in insert-borne and in normal ARG4 sequences, indicating that hotspot inactivation is not a consequence of changes in nucleosome positioning. However, a meiosis-specific increase in MNase hypersensitivity was no longer detected at the inactive insert-borne arg4 DSB site. Elimination of pBR322 sequences restored DSBs to the insert-borne arg4 promoter region and also restored the meiotic induction of MNase hypersensitivity. Thus, the meiotic induction of MNase hypersensitivity at the DSB sites is suppressed and activated in parallel to DSBs themselves, without changes in the underlying DNA sequence or nucleosome positioning. We suggest that meiosis-specific changes in chromatin at a DSB site are a signal reflecting a pivotal step in DSB formation.  (+info)

Expression of Escherichia coli K-12 arginine genes in Pseudomonas fluorescens. (3/123)

Escherichia coli argE and argH gene products were detected in Pseudomonas fluorescens argH122 carrying the E. coli F110 plasmid.  (+info)

Use of a recombination reporter insert to define meiotic recombination domains on chromosome III of Saccharomyces cerevisiae. (4/123)

In Saccharomyces cerevisiae, meiotic recombination is initiated by DNA double-strand breaks (DSBs). DSBs usually occur in intergenic regions that display nuclease hypersensitivity in digests of chromatin. DSBs are distributed nonuniformly across chromosomes; on chromosome III, DSBs are concentrated in two "hot" regions, one in each chromosome arm. DSBs occur rarely in regions within about 40 kb of each telomere and in an 80-kb region in the center of the chromosome, just to the right of the centromere. We used recombination reporter inserts containing arg4 mutant alleles to show that the "cold" properties of the central DSB-deficient region are imposed on DNA inserted in the region. Cold region inserts display DSB and recombination frequencies that are substantially less than those seen with similar inserts in flanking hot regions. This occurs without apparent change in chromatin structure, as the same pattern and level of DNase I hypersensitivity is seen in chromatin of hot and cold region inserts. These data are consistent with the suggestion that features of higher-order chromosome structure or chromosome dynamics act in a target sequence-independent manner to control where recombination events initiate during meiosis.  (+info)

(CA/GT)(n) microsatellites affect homologous recombination during yeast meiosis. (5/123)

One of the most common microsatellites in eukaryotes consists of tandem arrays of the dinucleotide GT. Although the study of the instability of such repetitive DNA has been extremely fruitful over the last decade, no biological function has been demonstrated for these sequences. We investigated the genetic behavior of a region of the yeast Saccharomyces cerevisiae genome containing a 39-CA/GT dinucleotide repeat sequence. When the microsatellite sequence was present at the ARG4 locus on homologous chromosomes, diploid cells undergoing meiosis generated an excess of tetrads containing a conversion of the region restricted to the region of the microsatellite close to the recombination-initiation double-strand break. Moreover, whereas the repetitive sequence had no effect on the frequency of single crossover, its presence strongly stimulated the formation of multiple crossovers. The combined data strongly suggest that numerous recombination events are restricted to the initiation side of the microsatellite as though progression of the strand exchange initiated at the ARG4 promoter locus was impaired by the repetitive sequence. This observation corroborates in vitro experiments that demonstrated that RecA-promoted strand exchange is inhibited by CA/GT dinucleotide tracts. Surprisingly, meiotic instability of the microsatellite was very high (>0.1 alterations per tetrad) in all the spores with parental and recombinant chromosomes.  (+info)

Guanidine hydrochloride induced reversible dissociation and denaturation of duck delta2-crystallin. (6/123)

The tetrameric delta2-crystallin from duck lens exhibits a reversible dissociation-denaturation process in solutions containing guanidine hydrochloride (GdnHCl). Sigmoidal or biphasic curves for the dissociation/denaturation processes, obtained using different methods of structural analysis, as a function of GdnHCl concentration were not coincidental with each other. delta2-crystallin in 0.91 M GdnHCl existed primarily as a monomer, which had no endogenous argininosuccinate lyase activity. After dilution of the GdnHCl-treated protein, the monomers reassociated into tetramers with concomitant recovery of enzyme activity. The sigmoidal recovery of enzyme activity demonstrates a cooperative hysteretic reactivation process. When the concentration of GdnHCl was higher than 1.2 M, various partially unfolded soluble forms of delta2-crystallin were produced from the dissociated monomers as shown by size-exclusion chromatography. The formation of a partially unfolded intermediate during the dissociation-denaturation process is proposed.  (+info)

Regulation of diaphragmatic nitric oxide synthase expression during hypobaric hypoxia. (7/123)

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)

Accumulation of arginine precursors in Escherichia coli: effects on growth, enzyme repression, and application to the forward selection of arginine auxotrophs. (8/123)

The accumulation or ornithine, citrulline, and possibly acetylornithine by Escherichia coli K-12 arginineless mutants provided with acetylarginine as source of arginine causes severe growth inhibition. This occurs under conditions where comparable derivatives of E. coli W (Bollon and Vogel, 1973) show little or no growth inhibition. The same conditions, which have been reported to cause noncorrelative synthesis of acetylornithinase and argininosuccinase in E. coli W (Bollon and Vogel, 1973), do not alter the correlative pattern of enzyme synthesis observed in E. coli K-12. Moreover, previously reported effects of ornithine and citrulline on repression of the arginine regulon in E. coli W are not observed in the K-12 strains examined. The bearing of these observations on possible differences between the mechanism of enzyme repression operating in the two types of strains cannot yet be fully evaluated; it is, however, clear that considerable care should be exercised before extrapolating the results obtained with one type of strain to the other one. The particularly strong inhibition of acetylarginine utilization exerted by ornithine in E. coli K-12 allows the forward selection of several classes of arginine auxotrophs from strains deficient in carbamoylphosphate biosynthesis and thus capable of ornithine accumulation. Possible applications of this technique to the genetic analysis of the bipolar argECBH operon are discussed.  (+info)

TY - JOUR. T1 - Requirement of argininosuccinate lyase for systemic nitric oxide production. AU - Erez, Ayelet. AU - Nagamani, Sandesh C.S.. AU - Shchelochkov, Oleg A.. AU - Premkumar, Muralidhar H.. AU - Campeau, Philippe M.. AU - Chen, Yuqing. AU - Garg, Harsha K.. AU - Li, Li. AU - Mian, Asad. AU - Bertin, Terry K.. AU - Black, Jennifer O.. AU - Zeng, Heng. AU - Tang, Yaoping. AU - Reddy, Anilkumar K.. AU - Summar, Marshall. AU - OBrien, William E.. AU - Harrison, David G.. AU - Mitch, William E.. AU - Marini, Juan C.. AU - Aschner, Judy L.. AU - Bryan, Nathan S.. AU - Lee, Brendan. PY - 2011/12/1. Y1 - 2011/12/1. N2 - Nitric oxide (NO) is crucial in diverse physiological and pathological processes. We show that a hypomorphic mouse model of argininosuccinate lyase (encoded by Asl) deficiency has a distinct phenotype of multiorgan dysfunction and NO deficiency. Loss of Asl in both humans and mice leads to reduced NO synthesis, owing to both decreased endogenous arginine synthesis and an ...
TY - JOUR. T1 - Argininosuccinate lyase interacts with cyclin A2 in cytoplasm and modulates growth of liver tumor cells. AU - Hung, Yu Hsuan. AU - Huang, Hau Lun. AU - Chen, Wei Ching. AU - Yen, Meng Chi. AU - Cho, Chien Yu. AU - Weng, Tzu Yang. AU - Wang, Chih Yang. AU - Chen, Yi Ling. AU - Chen, Li Tzong. AU - Lai, Ming Derg. PY - 2017/2. Y1 - 2017/2. N2 - Arginine is a critical amino acid in specific cancer types including hepatocellular carcinoma (HCC) and melanoma. Novel molecular mechanisms and therapeutic targets in arginine metabolism-mediated cancer formation await further identification. Our laboratory has previously demonstrated that arginine metabolic enzyme argininosuccinate lyase (ASL) promoted HCC formation in part via maintenance of cyclin A2 protein expression and arginine production for channeling to nitric oxide synthase. In this study, we investigated the mechanism by which ASL regulates cyclin A2 expression. We found that ASL interacted with cyclin A2 in HCC cells and the ...
p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.,/p> ,p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.,/p> ,p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).,/p> ,p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x,sup>64,/sup> + x,sup>4,/sup> + x,sup>3,/sup> + x + 1. The algorithm is described in the ISO 3309 standard. ,/p> ,p class=publication>Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.,br /> ,strong>Cyclic redundancy and other checksums,/strong>,br /> ,a href=http://www.nrbook.com/b/bookcpdf.php>Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993),/a>),/p> Checksum:i ...
ID ARLY_AGRVS Reviewed; 467 AA. AC B9JTJ2; DT 28-JUL-2009, integrated into UniProtKB/Swiss-Prot. DT 24-MAR-2009, sequence version 1. DT 25-OCT-2017, entry version 54. DE RecName: Full=Argininosuccinate lyase {ECO:0000255,HAMAP-Rule:MF_00006}; DE Short=ASAL {ECO:0000255,HAMAP-Rule:MF_00006}; DE EC=4.3.2.1 {ECO:0000255,HAMAP-Rule:MF_00006}; DE AltName: Full=Arginosuccinase {ECO:0000255,HAMAP-Rule:MF_00006}; GN Name=argH {ECO:0000255,HAMAP-Rule:MF_00006}; GN OrderedLocusNames=Avi_4017; OS Agrobacterium vitis (strain S4 / ATCC BAA-846) (Rhizobium vitis OS (strain S4)). OC Bacteria; Proteobacteria; Alphaproteobacteria; Rhizobiales; OC Rhizobiaceae; Rhizobium/Agrobacterium group; Agrobacterium. OX NCBI_TaxID=311402; RN [1] RP NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]. RC STRAIN=S4 / ATCC BAA-846; RX PubMed=19251847; DOI=10.1128/JB.01779-08; RA Slater S.C., Goldman B.S., Goodner B., Setubal J.C., Farrand S.K., RA Nester E.W., Burr T.J., Banta L., Dickerman A.W., Paulsen I., RA Otten L., Suen G., ...
SWISS-MODEL Repository entry for B3ECP3 (ARLY_CHLL2), Argininosuccinate lyase. Chlorobium limicola (strain DSM 245 / NBRC 103803 / 6330)
You ever have one of those months? I feel like I have been at the vet every other day for the last month. New pup visit with poop samples and
So I asked Chases PCs office to send the records. I inquired as to how the records would be sent, confirmed it was with an overnight service with tracking info, and was told the records would go out the next day. When I called the next day to confirm, they informed me they did in fact send the records that morning but it was via regular mail because it would have been a $35 charge for them to FedEx them. Hubs and I were not too pleased (call us crazy, but were not fans of medical records and/or personal identifying information floating around the USPS without any method of confirming delivery). I told the office I was NOT pleased that they made the decision for us concerning the $35 charge. I couldnt fathom why they couldnt pick up the phone and call me to ask what it was I wanted to do. Had they done so, I wouldve asked them to hold the copy of the records, had hubs pick them up on his way home, and send them ourselves via UPS (for waaaaaay less than $35!). But alas, it was too late. They ...
My responds - of course, I was mad and a bit confused. To clear my thought, I started to dig lot of journals regarding the chemotherapy regiment for GTD and the GTD itself. Found out that 5% of patients that undergo the MTX-FA will start to develop resistant towards the drugs. Argh… (again I considered myself as lucky-one to be in the statistical data ...
Argh! Im a week overdue with, what is predicted to be a BIG Bubba (no.2) & going for a sweep tomorrow. I just wondered, in your collective experienc
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 ...
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. ...
context: https://springernature.github.io/scigraph/jsonld/sgcontext.json, about: [ { id: http://purl.org/au-research/vocabulary/anzsrc-for/2008/0604, inDefinedTermSet: http://purl.org/au-research/vocabulary/anzsrc-for/2008/, name: Genetics, type: DefinedTerm }, { id: http://purl.org/au-research/vocabulary/anzsrc-for/2008/06, inDefinedTermSet: http://purl.org/au-research/vocabulary/anzsrc-for/2008/, name: Biological Sciences, type: DefinedTerm }, { inDefinedTermSet: https://www.nlm.nih.gov/mesh/, name: Arginine, type: DefinedTerm }, { inDefinedTermSet: https://www.nlm.nih.gov/mesh/, name: Argininosuccinate Lyase, type: DefinedTerm }, { inDefinedTermSet: https://www.nlm.nih.gov/mesh/, name: Argininosuccinate Synthase, type: DefinedTerm }, { inDefinedTermSet: https://www.nlm.nih.gov/mesh/, name: Bacterial Proteins, type: DefinedTerm }, { inDefinedTermSet: https://www.nlm.nih.gov/mesh/, name: Biosynthetic ...
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. ...
ID ARLY_METST Reviewed; 465 AA. AC Q2NGN7; DT 27-JUN-2006, integrated into UniProtKB/Swiss-Prot. DT 07-FEB-2006, sequence version 1. DT 25-OCT-2017, entry version 79. DE RecName: Full=Argininosuccinate lyase {ECO:0000255,HAMAP-Rule:MF_00006}; DE Short=ASAL {ECO:0000255,HAMAP-Rule:MF_00006}; DE EC=4.3.2.1 {ECO:0000255,HAMAP-Rule:MF_00006}; DE AltName: Full=Arginosuccinase {ECO:0000255,HAMAP-Rule:MF_00006}; GN Name=argH {ECO:0000255,HAMAP-Rule:MF_00006}; GN OrderedLocusNames=Msp_0618; OS Methanosphaera stadtmanae (strain ATCC 43021 / DSM 3091 / JCM 11832 / OS MCB-3). OC Archaea; Euryarchaeota; Methanobacteria; Methanobacteriales; OC Methanobacteriaceae; Methanosphaera. OX NCBI_TaxID=339860; RN [1] RP NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]. RC STRAIN=ATCC 43021 / DSM 3091 / JCM 11832 / MCB-3; RX PubMed=16385054; DOI=10.1128/JB.188.2.642-658.2006; RA Fricke W.F., Seedorf H., Henne A., Kruer M., Liesegang H., RA Hedderich R., Gottschalk G., Thauer R.K.; RT The genome sequence of ...
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
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 ...
Occurs when wild type phenotype is restored in an F1 individual made by crossing two independent mutants, carrying different heteroalleles
My scalp is itching and bumpy! I usually use Philip Kingsley itchy flaky shampoo and that seems to sort it out. But its so expensive. [sad] - does
Argh! First, the morning starts off when Im on my new diet/exercise program (oh, help…) and then you make me cry with the doggie video….. And now I have a combination of the slinky tune and the log tune from ren and stimpy stuck in my head….. @ Samantha, that popped in my head too! And it wont go away!!. ...
And, yes, the ultra sound showed that I had an enlarged thyroid with nodules. Could this possibly be the cause of my chronic cough that has progressively gotten worse over the past 7 or 8 years? At first it looked like the cough was solved. But gradually over the month or so since the surgery, Ive started coughing again. Not as much, but its still there. ARGH! Hope I didnt go through this for nothing ...
And, yes, the ultra sound showed that I had an enlarged thyroid with nodules. Could this possibly be the cause of my chronic cough that has progressively gotten worse over the past 7 or 8 years? At first it looked like the cough was solved. But gradually over the month or so since the surgery, Ive started coughing again. Not as much, but its still there. ARGH! Hope I didnt go through this for nothing ...
Yes you did!. No I didnt!. It was obviously you, who else would be so clumsy and careless?!. (sound of a door slamming). See Im right! Childish fool! Cant get any sense into your thick head! Argh!! Taeyeon yelled. He had been arguing with his younger brother Taejin the whole night over a trivial matter that grew out of proportion. Feeling very frustrated, he decided to get out of the house to cool himself down.. Grabbing a few items from his room, Taeyeon told his mother he would be staying over at a friends house tonight before he stormed out of the door.. ~*~*~*~. Ping. A tiny stone bounced off the window. Taeyeon waited to see if there was any movement. After two minutes, there was none besides the rustling of the leaves in the light breeze. He picked up another small stone and threw it against the window.. Ping. Nothing. Anxious, Taeyeon pulled out his mobile phone from his back pocket to type a message. He sat cross legged on the ground, with his back against the door of the ...
*snip* Argh. Whilst im not a fan of throwing the word innovation around, i feel just the same way about... authenticity or somewhat (at least in...
Broad said: Like I said, my feelings are complicated on the matter, so ... Im interested, however, in Her Highness thoughts on… ...[go].. Caterina said: ARGH!!! Not to deny you your goddess-given right of reflections and wishing what might-have-beens, but this guy was straight up… ...[go].. Wholovesya? said: By the by, guess who was most nasty about the charitable giving? The frigging church. My church and my moms… ...[go].. Wholovesya? said: By the by, Im not the only one I know. I have friends who work at soup kitchens because theyre… ...[go].. Wholovesya? said: As you know, I was a voyeur to the beginning of this, and I was loving your comment! I have… ...[go].. ...
Broad said: Like I said, my feelings are complicated on the matter, so ... Im interested, however, in Her Highness thoughts on… ...[go].. Caterina said: ARGH!!! Not to deny you your goddess-given right of reflections and wishing what might-have-beens, but this guy was straight up… ...[go].. Wholovesya? said: By the by, guess who was most nasty about the charitable giving? The frigging church. My church and my moms… ...[go].. Wholovesya? said: By the by, Im not the only one I know. I have friends who work at soup kitchens because theyre… ...[go].. Wholovesya? said: As you know, I was a voyeur to the beginning of this, and I was loving your comment! I have… ...[go].. ...
Maaheimo, H., Fiaux, J., Cakar, Z. P., Bailey, J. E., Sauer, U. & Szyperski, T., 2001, In : FEBS Journal. 268, 8, p. 2464-2479 16 p.. Research output: Contribution to journal › Article › Scientific › peer-review ...
Treatment may require the coordinated efforts of a team of specialists. Pediatricians, neurologists, geneticists, dieticians, and physicians who are familiar with metabolic disorders may need to work together to ensure a comprehensive approach to treatment. Occupational, speech language, and physical therapists may be needed to treat children with developmental disabilities. Genetic counseling is recommended for affected individuals and their families.. The treatment of argininosuccinic aciduria is aimed at preventing excessive ammonia from being formed or from removing excessive ammonia during a hyperammonemic episode. Long-term therapy combines dietary restrictions and the stimulation of alternative methods of converting and excreting nitrogen from the body (alternative pathways therapy).. Dietary restrictions in individuals with argininosuccinic aciduria are aimed at limiting the amount of protein intake to avoid the development of excess ammonia. However, enough protein must be taken in by ...
Argininosuccinate lyase deficiency (ASLD; also known as argininosuccinic aciduria) is the second most common urea cycle disorder (UCD) and accounts for 15-20% of all disorders of ureagenesis. Individuals with ASLD can have unique clinical and physiologic characteristics as compared to other UCDs. Previous work from the members of the UCDC have shown that in spite of having fewer episodes of hyperammonemia as compared to those with proximal blockade of the urea cycle, individuals with ASLD can develop intellectual and learning disabilities. Neurocognitive deficits have been observed even in individuals without any documented hyperammonemia. Furthermore, hepatic abnormalities including hepatomegaly, hepatic injury, fibrosis and even frank cirrhosis, and vascular issues like hypertension are well known in the disorder. Previous work from the members of the UCDC has demonstrated a tissue- and molecular-specific role for ASL in the generation of NO. ASL is not only required for the synthesis of ...
Argininosuccinic Aciduria; Arginino Succinase Deficiency. On-line free medical diagnosis assistant. Ranked list of possible diseases from either several symptoms or a full patient history. A similarity measure between symptoms and diseases is provided.
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 ...
In the linear pathway (Figure 1A), GLU is converted to acetylglutamate (Ac-GLU) by N-acetylglutamate synthase (NAGS, encoded by argA) which is inhibited by ARG through negative feedback regulation [36],[39]. Sequential catalytic reactions catalyzed by the next three enzymes, N-acetylglutamate kinase (NAGK, encoded by argB), N-acetylglutamate semialdehyde dehydrogenase (encoded by argC) and N-acetylornithine transaminase (encoded by argD), which are common in the three pathways (Figure 1), yield N-acetylornithine (Ac-ORN) [34]. The next step, which distinguishes the linear pathway from the other two pathways, is deacetylation of Ac-ORN by AOase to yield ORN [40],[41]. The next and final steps are carried out by ornithine carbamoyltransferase (OTC or OTCase, encoded by argF), argininosuccinate synthase (encoded by argG) and argininosuccinate lyase (encoded by argH), which finally yield ARG [35]. This pathway has been found in a few species such as Myxococcus xanthus [41] and E. coli [36].. In many ...
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
Kleijer WJ et al. (2002) Clinical, enzymatic, and molecular genetic characterization of a biochemical variant type of argininosuccinic aciduria: prenatal and postnatal diagnosis in five unrelated families.. [^] ...
p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.,/p> ,p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.,/p> ,p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).,/p> ,p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x,sup>64,/sup> + x,sup>4,/sup> + x,sup>3,/sup> + x + 1. The algorithm is described in the ISO 3309 standard. ,/p> ,p class=publication>Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.,br /> ,strong>Cyclic redundancy and other checksums,/strong>,br /> ,a href=http://www.nrbook.com/b/bookcpdf.php>Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993),/a>),/p> Checksum:i ...
Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. Pubmed: 19212411 ...
1KH2: Crystal structure of argininosuccinate synthetase from Thermus thermophilus HB8. Structural basis for the catalytic action.
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 ...
Complete information for ASS1P12 gene (Pseudogene), Argininosuccinate Synthetase 1 Pseudogene 12, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
Complete information for ASS1P14 gene (Pseudogene), Argininosuccinate Synthetase 1 Pseudogene 14, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
SWISS-MODEL Template Library (SMTL) entry for 1kh3.1. Crystal Structure of Thermus thermophilus HB8 Argininosuccinate Synthetase in complex with inhibitor
I started blogging years ago as a weight loss tool. I lost and gained a lot of weight over the years but more importantly I gained a lot of friends who still read my blog! Thru my 12 years of blogging, life has changed quite a bit for me! Im now disabled, living with my parents and one of my adult sons and Im now Nana to Cosette and Oliver, born 3 months apart. Life looks different for me now so I wanted my blog to look different. Welcome to the Nana Life. ...
David is a young hobby astronomer, who one day accidentally shots down an alien spacecraft by his self-made model rocket. In just that moment the UFO crashes in front of him his own rocket knocks him out. He awakes on a foreign farm and has to find out...
Then a few days later I want to do a show ip bgp hmm output not as expected. Right this time I twig it has to be one of those pesky VRFs getting in my way. So lets try doing show ip vrf abc wooh no bgp command. Okay lets try figure this out show ip ? and look through the options hmm nothing there so how about a show ip bgp ? hmm nothing there either. Argh so I am stumped how the heck do you do a show ip bgp when you have VRFs around? Time to prepare colleague for another stupid question by offering to make him a drink. Once drink has been made then I utilize my chance for another stupid question. Umm so you know these VRFs how do you do a show ip bgp turns out the answer is to do a show ip bgp vpnv4 vrf abc. So I have enough trouble with VRFs and then they go add the vpnv4 command before the vrf command. Guess I will get to that bit when I sort out multi-protocol BGP. Anyhow you will be pleased to know that I am getting on a lot better with VRFs now. However as with anything on this blog ...
I feel like one huge swollen tonsil. Like my eustachian tubes (etruscan tubes? I cant spell yoostayshun) are on fire. The back of my throat has rabid bats roosting in it, dropping guano....burning guano, down my esophagus. And to make it even more annoying, this has been slowly slowwwwwwly coming on for two damn weeks.…
I voted yesterday morning. Got my civic duty done. I love our little county... such sweet people, always friendly... everyone greets you with a smile and it makes every little errand or chore completely pleasant. I asked the election officials how many registered voters we have in our county... they said 14,000. So if that gives you an idea of what a small town this is... conversely... when we lived in Duval County, Florida... there are 1.1 million registered voters. This is why ... everywhere you go... you run into someone you know. If not for tourists, wed never have traffic. but we appreciate the tourists... they stimulate the economy, right ...
Scores of would-be swashbucklers found themselves in need of rescuing Saturday night, when their ship -- the Pirates Ransom -- ran aground in west Florida.
Have you ever had a day like this? Overslept. Didnt accomplish what I wanted. Things in the house broke. My phone kept sticking and not letting me type. ARGH! Drivers on the rainy roads drove crazy (that may have been because of my grumpy mood). I basically flailed on the edge of losing it all day long. Continue Reading…. ...
So glad she does not have the flu. I know the anxiety you are having. It sounds like A has a lot of extra health issues that make having the flu a lot of extra work and even more scary. Same with M. I am constantly questioning what I am doing in order to help her stay healthy. I cant keep her in a bubble but when it is all over her school I can keep her home. Does that do any good in the long run since these outbreaks could go on and then there are different viruses anyway that can send her into CVS and then to the hospital. Sigh. Argh. I go around and around about it. ...
And by that time, Im past him and spend the rest of my ride home going back and forth in this way. Im sick of it. Maybe one day I should just come stand next to you and do the same thing, or when I forget my wallet, ask you for some change. Argh.. 3. Apple basket Pirates. Back off. Seriously. If you understand that analogy, great. If not, and you want to, just ask. 4. Two dimensional thinkers. Im used to being around people who, when faced with a problem or something they dont understand, try and figure it out on their own before asking everyone around them to do it for them. Those people are three dimensional thinkers-I would even go so far as to say they are multi-dimensional thinkers. Two dimensional thinkers make zero effort to figure something out on their own and hand it off to other people. Oh. This doesnt work. Rather than making an effort to be intelligent and solve the problem myself, Ill just call ten other people and make them do it for me because Im intellectually lazy. Im ...
Hospital Escape : Argh, I dont like hospitals! Out, please! If only I wasnt drugged, this might be easier. And where are the nurses? Free Online Puzzle Games from AddictingGames
Argh! I hate when I hear something cool on the radio from the other room and bring up the playlist to see who it is and its Steve Reich ...
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Nagamani SC, Erez A, Lee B (May 2012). "Argininosuccinate lyase deficiency". Genetics in Medicine. 14 (5): 501-507. doi:10.1038 ...
"Gene sharing by delta-crystallin and argininosuccinate lyase". Proceedings of the National Academy of Sciences of the United ...
In humans, argininosuccinate lyase (ASL) is a homotetrameric enzyme that can undergo intragenic complementation. An ASL ... 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. ... "Mechanisms for intragenic complementation at the human argininosuccinate lyase locus". Biochemistry. 40 (51): 15581-90. doi: ...
In argininosuccinic aciduria, the enzyme argininosuccinate lyase, involved in the conversion of arginino succinate to arginine ... "Detection of neonatal argininosuccinate lyase deficiency by serum tandem mass spectrometry". J Inherit Metab Dis. 24 (3): 370-8 ...
... is a precursor to fumarate in the citric acid cycle via argininosuccinate lyase. Argininosuccinate ... The enzyme that catalyzes the reaction is argininosuccinate synthetase. ...
Ants quickly lost the ability to synthesize arginine by losing the argininosuccinate lyase gene, the final step in the arginine ...
... proline metabolism by the sequential action of the cytosolic enzymes argininosuccinate synthetase and argininosuccinate lyase. ... This is an energetically costly process, because for each molecule of argininosuccinate that is synthesized, one molecule of ...
... and argininosuccinate lyase (ASL). Elia, Ilaria; Broekaert, Dorien; Christen, Stefan; Boon, Ruben; Radaelli, Enrico; Orth, ... is then synthesized from citrulline in the urea cycle by the sequential action of the cytosolic enzymes argininosuccinate ...
... a Belgian air taxi and charter airline American Society of Lymphology Argininosuccinate lyase, an enzyme Arterial spin ...
1 carbamoyl phosphate synthetase I OTC Ornithine transcarbamoylase ASS argininosuccinate synthetase ASL argininosuccinate lyase ... This reaction is ATP dependent and is catalyzed by argininosuccinate synthetase. Argininosuccinate undergoes cleavage by ... The reactions of the urea cycle 1 L-ornithine 2 carbamoyl phosphate 3 L-citrulline 4 argininosuccinate 5 fumarate 6 L-arginine ... Deficiency of argininosuccinic acid lyase) Argininemia (Deficiency of arginase) Hyperornithinemia, hyperammonemia, ...
... with those from birds and reptiles related to lactate dehydrogenase and argininosuccinate lyase, those of mammals to alcohol ...
Argininosuccinate synthetase and argininosuccinate lyase recycle citrulline, a byproduct of nitric oxide production, into ... 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 ... Expression of argininosuccinate synthetase in the intestines ceases after two to three years of life. It is thought that ...
Disorders Overview GeneReviews/NCBI/NIH/UW entry on Argininosuccinate Lyase Deficiency OMIM entries on Argininosuccinate Lyase ... Argininosuccinate lyase is an intermediate enzyme in the urea synthesis pathway and its function is imperative to the ... "ASL gene argininosuccinate lyase". NIH. U.S. Department of Health & Human Services. 2007. Jack, JJB (1982). "Actions of ammonia ... ASL (argininosuccinate lyase, also known as argininosuccinase) is an enzyme that catalyzes the reversible breakdown of ...
... argininosuccinate lyase), which catalyzes the formation of arginine and fumarate from argininosuccinate, the last step in the ... Fumarate lyase is a substrate of the lyase class of enzymes. It been shown to share a short conserved sequence around a ... Aspartate ammonia-lyase, EC 4.3.1.1 (aspartase), which catalyzes the reversible conversion of aspartate to fumarate and ammonia ... Delta-crystallin shares around 90% sequence identity with arginosuccinate lyase, showing that it is an example of a 'hijacked' ...
... amidine-lyases MeSH D08.811.520.232.300.200 - adenylosuccinate lyase MeSH D08.811.520.232.300.400 - argininosuccinate lyase ... chondroitin lyases MeSH D08.811.520.241.700.350.500.500 - chondroitin abc lyase MeSH D08.811.520.241.700.512 - heparin lyase ... lyase MeSH D08.811.520.241.300 - hydro-lyases MeSH D08.811.520.241.300.050 - aconitate hydratase MeSH D08.811.520.241.300.050. ... oxo-acid-lyases MeSH D08.811.520.224.600.200 - anthranilate synthase MeSH D08.811.520.224.600.700 - isocitrate lyase MeSH ...
... argininosuccinate lyase EC 4.3.2.2: adenylosuccinate lyase EC 4.3.2.3: ureidoglycolate lyase EC 4.3.2.4: purine imidazole-ring ... pectin lyase EC 4.2.2.11: poly(a-L-guluronate) lyase EC 4.2.2.12: xanthan lyase EC 4.2.2.13: exo-(1-4)-α-D-glucan lyase EC 4.2. ... pectate lyase EC 4.2.2.3: poly(b-D-mannuronate) lyase EC 4.2.2.4: chondroitin ABC lyase EC 4.2.2.5: chondroitin AC lyase EC 4.2 ... oligogalacturonide lyase EC 4.2.2.7: heparin lyase EC 4.2.2.8: heparin-sulfate lyase EC 4.2.2.9: pectate disaccharide-lyase EC ...
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 ... including argininosuccinate synthetase) EC 6.4 includes ligases used to form carbon-carbon bonds EC 6.5 includes ligases used ...
EC 4.3.1 Phenylalanine ammonia-lyase (EC 4.3.1.24) Category:EC 4.4.1 Cystathionine gamma-lyase Cystathionine beta-lyase ... Argininosuccinate synthetase (EC 6.3.4.5) CTP synthase (EC 6.3.4.2) Pyruvate carboxylase (EC 6.4.1.1) Acetyl-CoA carboxylase ( ... lyase) ligase EC 6.2.1.23: Dicarboxylate-CoA ligase EC 6.2.1.24: Phytanate-CoA ligase EC 6.2.1.25: Benzoate-CoA ligase EC 6.2. ...
The following reaction step, catalyzed by the enzyme O-acetyl serine (thiol) lyase, replaces the acetyl group of O-acetyl-L- ... Then, the enzymes citrulline and argininosuccinate convert ornithine to arginine. There are two distinct lysine biosynthetic ... SAICAR lyase removes the carbon skeleton of the added aspartate, leaving the amino group and forming 5-aminoimidazole-4- ...
... is a rare disease caused by a deficiency in argininosuccinate synthetase, an enzyme involved in excreting excess nitrogen from ... argininosuccinic acid lyase deficiency, ornithine transcarbamylase deficiency, arginase deficiency, and N-Acetylglutamate ...
... lyase) ligase EC 6.2.1.23: dicarboxylate-CoA ligase EC 6.2.1.24: phytanate-CoA ligase EC 6.2.1.25: benzoate-CoA ligase EC 6.2. ... argininosuccinate synthase EC 6.3.4.6: urea carboxylase EC 6.3.4.7: ribose-5-phosphate-ammonia ligase EC 6.3.4.8: ...
... anemia due to Adenylosuccinate lyase deficiency Adie syndrome Adiposis dolorosa, aka Dercum's disease Adolescent benign focal ... II Arbovirosis Arc syndrome AREDYLD syndrome Argentine hemorrhagic fever Arginase deficiency Arginemia Argininosuccinate ...
Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. *Carbamoyl phosphate synthetase ...
Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. *Carbamoyl phosphate synthetase ...
EC4 Lyases (list). *EC5 Isomerases (list). *EC6 Ligases (list). *EC7 Translocases (list) ... Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. *Carbamoyl phosphate synthetase ...
Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. *Carbamoyl phosphate synthetase ...
Category:Lyases (EC 4) (Lyase)Edit. Category:EC 4.1 (carbon-carbon lyases)Edit. *Category:EC 4.1.1 *Ornithine decarboxylase (EC ... Argininosuccinate synthetase (EC 6.3.4.5). *CTP synthase (EC 6.3.4.2). Category:EC 6.4 (form carbon-carbon bonds)Edit. * ... 4 Category:Lyases (EC 4) (Lyase) *4.1 Category:EC 4.1 (carbon-carbon lyases) ... Category:EC 4.3 (carbon-nitrogen lyases)Edit. *Category:EC 4.3.1 *Phenylalanine ammonia-lyase (EC 4.3.1.24) ...
2-hydroxyphytanoyl-CoA lyase. *2-oxo-4-hydroxy-4-carboxy-5-ureidoimidazoline decarboxylase ... Argininosuccinate synthase. *Argonaute. *Argos (EGFR Inhibitor). *Aromatic L-amino acid decarboxylase. *Arrestin ...
EC4 Lyases (list). *EC5 Isomerases (list). *EC6 Ligases (list). *EC7 Translocases (list) ... Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. *Carbamoyl phosphate synthetase ...
Disorders Overview GeneReviews/NCBI/NIH/UW entry on Argininosuccinate Lyase Deficiency OMIM entries on Argininosuccinate Lyase ... Argininosuccinate lyase is an intermediate enzyme in the urea synthesis pathway and its function is imperative to the ... "ASL gene argininosuccinate lyase". NIH. U.S. Department of Health & Human Services. 2007. Jack, JJB (1982). "Actions of ammonia ... ASL (argininosuccinate lyase, also known as argininosuccinase) is an enzyme that catalyzes the reversible breakdown of ...
... lyase deficiency results in defective cleavage of ASA. This leads to an accumulation of ASA in cells and an excessive excretion ... encoded search term (Argininosuccinate Lyase (ASL) Deficiency) and Argininosuccinate Lyase (ASL) Deficiency What to Read Next ... Argininosuccinate Lyase (ASL) Deficiency. Updated: Jan 07, 2019 * Author: Karl S Roth, MD; Chief Editor: Maria Descartes, MD ... Argininosuccinate (ASA) lyase deficiency results in defective cleavage of ASA. This leads to an accumulation of ASA in cells ...
IPR009049 Argininosuccinate_lyase. IPR024083 Fumarase/histidase_N. IPR020557 Fumarate_lyase_CS. IPR000362 Fumarate_lyase_fam. ... IPR009049 Argininosuccinate_lyase. IPR024083 Fumarase/histidase_N. IPR020557 Fumarate_lyase_CS. IPR000362 Fumarate_lyase_fam. ... Belongs to the lyase 1 family. Argininosuccinate lyase subfamily.UniRule annotation. Manual assertion according to rulesi ... Argininosuccinate lyase (argH). This subpathway is part of the pathway L-arginine biosynthesis, which is itself part of Amino- ...
Molecular cloning of the ARG7 gene of Schizosaccharomyces pombe encoding argininosuccinate lyase. *Jacques E. Remacle1. , ... One colony was recovered which contained the YEp13 plasmid bearing a large insert complementing the argininosuccinate lyase ( ... Molecular cloning of the ARG7 gene of Schizosaccharomyces pombe encoding argininosuccinate lyase. Curr Genet 14, 381-385 (1988 ...
Preparation of recombinant argininosuccinate synthetase and argininosuccinate lyase: expression of the enzymes in rat tissues. ... Hyperammonemia: regulation of argininosuccinate synthetase and argininosuccinate lyase genes in aggregating cell cultures of ... Argininosuccinate synthetase and argininosuccinate lyase are localized around mitochondria: an immunocytochemical study. Cohen ... Insulin antagonism of dexamethasone-induced increase or argininosuccinate synthetase and argininosuccinate lyase activities in ...
Sequence analysis of the ARG7 gene of Schizosaccharomyces pombe coding for argininosuccinate lyase. Expression of the gene in ... Functional conservation between the argininosuccinate lyase of the archaeon Methanococcus maripaludis and the corresponding ... Sequence analysis of the ARG7 gene of Schizosaccharomyces pombe coding for argininosuccinate lyase. Expression of the gene in ... namely argininosuccinate lyase, PR-aminoimidazolesuccinocarboxamide synthase, and orotidine-5-phosphate decarboxylase, ...
Epigenetic status of argininosuccinate synthetase and argininosuccinate lyase modulates autophagy and cell death in ... 2013) Epigenetic status of argininosuccinate synthetase and argininosuccinate lyase modulates autophagy and cell death in ...
... is caused by a defect of the urea cycle enzyme argininosuccinate lyase (ASL) encoded by the ASL gene. Patients often present ... Argininosuccinate lyase deficiency (ASLD) is caused by a defect of the urea cycle enzyme argininosuccinate lyase (ASL) encoded ... Low prevalence of argininosuccinate lyase deficiency among inherited urea cycle disorders in Korea. Dahye Kim, Jung Min Ko, +4 ... Cloning and sequence analysis of cDNA for human argininosuccinate lyase.. William E Obrien, Roderick R Mcinnes, K. E. Kalumuck ...
This gene encodes a member of the lyase 1 family. The encoded protein forms a cytosolic homotetramer and primarily catalyzes ... Nitrogen metabolism; urea cycle; L-arginine and fumarate from (N(omega)-L-arginino)succinate: step 1/1. 1 ... Mutations in this gene result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase ... the reversible hydrolytic cleavage of argininosuccinate into arginine and fumarate, an essential step in the liver in ...
Requirement of argininosuccinate lyase for systemic nitric oxide production. In: Nature Medicine. 2011 ; Vol. 17, No. 12. pp. ... Requirement of argininosuccinate lyase for systemic nitric oxide production. Nature Medicine. 2011 Dec 1;17(12):1619-1626. ... Requirement of argininosuccinate lyase for systemic nitric oxide production. Ayelet Erez, Sandesh C.S. Nagamani, Oleg A. ... Requirement of argininosuccinate lyase for systemic nitric oxide production. / Erez, Ayelet; Nagamani, Sandesh C.S.; ...
Hu, Y., Rolfs, A., Bhullar, B., Murthy, T. V., Zhu, C., Berger, M. F., Camargo, A. A., Kelley, F., McCarron, S., Jepson, D., Richardson, A., Raphael, J., Moreira, D., Taycher, E., Zuo, D., Mohr, S., Kane, M. F., Williamson, J., Simpson, A., Bulyk, M. L., Harlow, E., Marsischky, G., Kolodner, R. D., LaBaer, J. (2007). "Approaching a complete repository of sequence-verified protein-encoding clones for Saccharomyces cerevisiae." Genome Res 17:536-543.17322287 ...
Caulobacter crescentus Argininosuccinate lyase (argH) datasheet and description hight quality product and Backed by our ... Caulobacter crescentus Argininosuccinate lyase (argH). Alternative name: Caulobacter crescentus Argininosuccinate lyase (argH) ... Recombinant Caulobacter crescentus Argininosuccinate lyase (argH). Alternative names: Argininosuccinate lyase, Arginosuccinase ... Argininosuccinate lyase(argH) is a recombinant protein expressed in Yeast ...
Argininosuccinase acid lyase explanation free. What is Argininosuccinase acid lyase? Meaning of Argininosuccinase acid lyase ... Looking for online definition of Argininosuccinase acid lyase in the Medical Dictionary? ... argininosuccinate lyase. (redirected from Argininosuccinase acid lyase) ar·gi·ni·no·suc·ci·nate ly·ase. (arji-ni-nō-sŭksi-nāt ... a href=https://medical-dictionary.thefreedictionary.com/Argininosuccinase+acid+lyase,argininosuccinate lyase,/a,. *Facebook ...
Argininosuccinate lyase is an essential regulator of systemic nitric oxide produc Lee, Brendan Hl Baylor College of Medicine, ... Argininosuccinate lyase is an essential regulator of systemic nitric oxide produc. Lee, Brendan / Baylor College of Medicine. ... Argininosuccinate lyase is an essential regulator of systemic nitric oxide produc. Lee, Brendan / Baylor College of Medicine. ... Argininosuccinate lyase is an essential regulator of systemic nitric oxide produc. Lee, Brendan / Baylor College of Medicine. ...
ASL: argininosuccinate lyase. *ASNS: asparagine synthetase (glutamine-hydrolyzing). *ASPA: aspartoacylase. *ASPM: abnormal ...
The ASL gene provides instructions for making the enzyme argininosuccinate lyase. Learn about this gene and related health ... in the argininosuccinate lyase enzyme. Mutations in the ASL gene may result in an argininosuccinate lyase enzyme that is ... If the argininosuccinate lyase enzyme is misshapen or missing, it cannot fulfill its role in the urea cycle. Arginine is not ... Human argininosuccinate lyase: a structural basis for intragenic complementation. Proc Natl Acad Sci U S A. 1997 Aug 19;94(17): ...
argininosuccinate lyase. ASS. argininosuccinate synthase. CPA. N-carbamoylputrescine amidohydrolase. CPS. carbamoylsynthetase. ...
Argininosuccinate lyase. Shigella boydii serotype 18 (strain CDC 3083-94 / BS512) ... Argininosuccinate lyase UniProtKBInterProInteractive Modelling. 457 aa; Sequence (Fasta) Identical sequences: Shigella flexneri ...
ASL deficiency -- Argininosuccinate lyase. *ASS1 deficiency -- Argininosuccinate synthase 1. *Citrin deficiency -- Citrin ...
Review Argininosuccinate Lyase Deficiency[GeneReviews®. 1993]. Review Argininosuccinate Lyase Deficiency. Nagamani SCS, Erez A ...
Fumarate lyase, N-terminal. IPR022761PF00206. 372-438. Argininosuccinate lyase, C-terminal. IPR029419PF14698. ... Argininosuccinate lyase. Chlorobium limicola (strain DSM 245 / NBRC 103803 / 6330) ...
inherited deficiency of argininosuccinate lyase * inherited carbamoyl phosphate synthetase deficiency * citrullinemia * ...
... which is then taken up into the kidney and converted by argininosuccinate synthase (ASS) and argininosuccinate lyase (ASL) into ... Argininosuccinate lyase (ASL) enzyme was also down-regulated. The kidney plays a key role in arginine metabolism. Renal l- ...
Argininosuccinate Lyase Deficiency Urea Cycle Disorder Urea Cycle Disorders, Inborn Argininosuccinic Aciduria Dietary ... Patients with argininosuccinate lyase deficiency (ASLD) will be randomly assigned to receive either a nitric oxide dietary ... Argininosuccinate lyase deficiency (ASLD; also known as argininosuccinic aciduria) is the second most common urea cycle ... Nagamani SCS, Erez A, Lee B. Argininosuccinate Lyase Deficiency. 2011 Feb 3 [updated 2012 Feb 2]. In: Adam MP, Ardinger HH, ...
Hung, Y. H., Huang, H. L., Chen, W. C., Yen, M. C., Cho, C. Y., Weng, T. Y., ... Lai, M. D. (2017). Argininosuccinate lyase ... Argininosuccinate lyase interacts with cyclin A2 in cytoplasm and modulates growth of liver tumor cells. In: Oncology Reports. ... Argininosuccinate lyase interacts with cyclin A2 in cytoplasm and modulates growth of liver tumor cells. Oncology Reports. 2017 ... Argininosuccinate lyase interacts with cyclin A2 in cytoplasm and modulates growth of liver tumor cells. / Hung, Yu Hsuan; ...
Review Argininosuccinate Lyase Deficiency[GeneReviews®. 1993]. Review Argininosuccinate Lyase Deficiency. Nagamani SCS, Erez A ... Argininosuccinate synthase enzyme activity can be measured in uncultured fetal tissue obtained by chorionic villus sampling ( ... Abnormal gene product. The argininosuccinate synthase enzyme is inactive or absent. Mutated ASS with abnormal KM (Michaelis ... Argininosuccinate synthase (ASS) enzyme activity. Incorporation of radiolabeled citrulline into argininosuccinic acid is ...
Argininosuccinate Lyase (ASL) Deficiency * Carbamoyl Phosphate Synthetase (CPS) Deficiency * N-Acetylglutamate Synthetase ... Compound 5 is fumaric acid generated in the reaction that converts ASA to arginine (6), which is mediated by ASA lyase. ...
... argininosuccinate synthetase deficiency (citrullinemia); argininosuccinate lyase (ASL) deficiency; arginase deficiency ( ...
Argininosuccinate lyase. Serum. 0-4 U/dL. 10. 0-40 U/L. ...
Argininosuccinate Lyase 4.3.2.1. (R)-Aromatic Alcohol Dehydrogenase, NADP+ Dependent (EC 1.1.1.2) 1.1.1.2. ...
  • Our laboratory has previously demonstrated that arginine metabolic enzyme argininosuccinate lyase (ASL) promoted HCC formation in part via maintenance of cyclin A2 protein expression and arginine production for channeling to nitric oxide synthase. (ncku.edu.tw)
  • 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)
  • De novo synthesized citrulline could also be converted back to arginine in the kidneys by argininosuccinate synthase and argininosuccinate lyase ( 12 ) in an attempt to stabilize circulating and tissue concentrations of arginine. (diabetesjournals.org)
  • The ASS1 gene provides instructions for making argininosuccinate synthase 1, which is responsible for the third step of the urea cycle. (thefreedictionary.com)
  • We have studied the combination of L-CAV with VIN and PTX in three cancer cell lines representing cells with high argininosuccinate synthase (ASS) expression (HeLa) and cells with low ASS expression (Hep G2 and SK-HEP-1 cells). (thefreedictionary.com)
  • Cit is transported out from mitochondrion and bound to aspartate by argininosuccinate synthase, and aspartate is provided by the mitochondrial transporter citrin. (thefreedictionary.com)
  • Neuronal and glial coexpression of argininosuccinate synthetase and inducible nitric oxide synthase in Alzheimer disease. (thefreedictionary.com)
  • argininosuccinate synthase (nine peptides), elongation factor 1-alpha (two peptides), alanine aminotransferase2 (two peptides), and 26S protease regulatory subunit6B homolog (five peptides, also found in other spots) (Table 1, peptide data not shown). (thefreedictionary.com)
  • 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)
  • 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)
  • These cancers have a high incidence of deficiencies of the arginine synthesis enzyme argininosuccinate synthetase (ASS) due to hypermethylation at the promoter of the gene that codes for ASS ( ASS1 ). (drugs.com)
  • Mutations in the Human Argininosuccinate Synthetase (ASS1) Gene, Impact on Patients, Common Changes, and Structural Considerations. (uzh.ch)
  • Argininosuccinate lyase deficiency (ASLD) is caused by a defect of the urea cycle enzyme argininosuccinate lyase (ASL) encoded by the ASL gene. (semanticscholar.org)
  • The ASL gene provides instructions for making the enzyme argininosuccinate lyase. (medlineplus.gov)
  • Arginosuccinase, EC:4.3.2.1 (argininosuccinate lyase), which catalyses the formation of arginine and fumarate from argininosuccinate, the last step in the biosynthesis of arginine. (ebi.ac.uk)
  • Mutations and polymorphisms in the human argininosuccinate lyase (ASL) gene. (semanticscholar.org)
  • Argininosuccinate lyase deficiency: evidence for heterogeneous structural gene mutations by immunoblotting. (semanticscholar.org)
  • Mutations in this gene result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase deficiency. (nih.gov)
  • Other mutations replace one protein building block (amino acid) with another amino acid in the argininosuccinate lyase enzyme. (medlineplus.gov)
  • In people of Arab ancestry, two common mutations replace the amino acid glutamine with a premature stop signal at position 116 (written as Gln116Ter or Q116*) or position 354 (written as Gln354Ter or Q354*) in the argininosuccinate lyase enzyme. (medlineplus.gov)
  • Mutations in the ASL gene may result in an argininosuccinate lyase enzyme that is unstable, misshapen, or quickly broken down. (medlineplus.gov)
  • Argininosuccinic aciduria is caused by mutations in the argininosuccinate lyase gene (ASL) which catalyzes the cleavage of the argininosuccinate to fumarate and arginine. (renalandurologynews.com)
  • Identification of mutations in Malaysian patients with argininosuccinate lyase (ASL) deficiency. (annals.org)
  • specific complementation of argininosuccinate lyase ( arg H) mutations. (springer.com)
  • Delta-crystallin shares around 90% sequence identity with arginosuccinate lyase, showing that it is an example of a 'hijacked' enzyme - accumulated mutations have, however, rendered the protein enzymatically inactive. (ebi.ac.uk)
  • Kinetic mutations in argininosuccinate synthetase deficiency: characterisation and in vitro correction by substrate supplementation. (uzh.ch)
  • Argininosuccinic Acid Lyase Deficiency Missed by Newborn Screen. (semanticscholar.org)
  • Argininosuccinate lyase deficiency-argininosuccinic aciduria and beyond. (semanticscholar.org)
  • This renewal application focuses on studying the efficacy of nitric oxide (NO) supplementation as a treatment for the vascular complications in argininosuccinic aciduria (ASA) caused by deficiency of argininosuccinate lyase (ASL), the second most common urea cycle disorder and a human model of nitric oxide (NO) deficiency. (grantome.com)
  • This project will test whether supplementation with a nutriceutical source of nitric oxide (inorganic nitrite) can be used to treat the vascular dysfunction in patients with argininosuccinic aciduria due to deficiency of argininosuccinate lyase (ASL). (grantome.com)
  • Tanaka T, Nagao M, Mori T, Tsutsumi H. A novel stop codon mutation (X465Y) in the argininosuccinate lyase gene in a patient with argininosuccinic aciduria. (medlineplus.gov)
  • Citrulline level is normal in arginine deficiency and is markedly elevated in argininosuccinic lyase deficiency, usually 100 times the upper limit of the normal range. (renalandurologynews.com)
  • Argininosuccinic acid in plasma and urine permits the specific diagnosis of Argininosuccinic lyase deficiency. (renalandurologynews.com)
  • Argininosuccinic lyase is involved in the conversion of the citrulline to arginine in other tissues. (renalandurologynews.com)
  • Understanding the role of argininosuccinate lyase transcript variants in the clinical and biochemical variability of the urea cycle disorder argininosuccinic aciduria. (uzh.ch)
  • While ASS catalyzes the formation of argininosuccinate from citrulline and aspartate, ASL breaks the newly formed argininosuccinate into L-arginine and fumarate. (wikipedia.org)
  • The third step is catalyzed by an enzyme called argininosuccinate synthetase, which uses citrulline and ATP to form a citrullyl-AMP intermediate, which reacts with an amino group from aspartate to produce argininosuccinate. (news-medical.net)
  • Aspartate ammonia-lyase, EC:4.3.1.1 (aspartase), which catalyses the reversible conversion of aspartate to fumarate and ammonia. (ebi.ac.uk)
  • Additional galU genes were found adjacent to the gene argH encoding argininosuccinate lyase in the sequenced genomes from isolates a275, 29031, H-ASH/87, and H-ZANE/83, and although the genes were conserved, they were not identical to the galU genes found within the cps locus. (thefreedictionary.com)
  • These enzymes are carbamoyl phosphate synthetase 1 (CPS1), ornithine transcarbamylase (OTC), argininosuccinate synthetase (ASS), argininosuccinate lyase (ASL), arginase and N-acetylglutamate synthetase. (thefreedictionary.com)
  • ASL (argininosuccinate lyase, also known as argininosuccinase) is an enzyme that catalyzes the reversible breakdown of argininosuccinate (ASA) producing the amino acid arginine and dicarboxylic acid fumarate. (wikipedia.org)
  • The enzyme's cleavage of the argininosuccinate, to form fumarate and arginine, occurs through an E1cb elimination reaction. (wikipedia.org)
  • ASL, δ-crystallin, class II fumarase, aspartase, adenylosuccinase lyase, and 3-carboxy-cis and cis-muconate lactonizing enzyme are all members of the same homotetrameric superfamily of enzymes, in which most catalyze the same type of elimination reactions where a C-O or C-N bond is broken and fumarate is released as a product. (wikipedia.org)
  • The encoded protein forms a cytosolic homotetramer and primarily catalyzes the reversible hydrolytic cleavage of argininosuccinate into arginine and fumarate, an essential step in the liver in detoxifying ammonia via the urea cycle. (nih.gov)
  • The fourth step involves the cleavage of argininosuccinate to form fumarate and arginine. (news-medical.net)
  • A number of enzymes, belonging to the lyase class, for which fumarate is a substrate, have been shown to share a short conserved sequence around a methionine which is probably involved in the catalytic activity of this type of enzymes [ PMID: 3282546 ]. (ebi.ac.uk)
  • Adenylosuccinase, EC:4.3.2.2 (adenylosuccinate lyase) [ PMID: 1574589 ], which catalyses the eighth step in the de novo biosynthesis of purines, the formation of 5'-phosphoribosyl-5-amino-4-imidazolecarboxamide and fumarate from 1-(5-phosphoribosyl)-4-(N-succino-carboxamide). (ebi.ac.uk)
  • Increased levels of blood citrulline and argininosuccinate are also seen in cases of citrullinemia. (news-medical.net)
  • Argininosuccinate (ASA) lyase deficiency is the second most common urea cycle disorder in the pediatric population. (stanford.edu)
  • ASL catalyzes the fourth step in the cycle, following the action of argininosuccinate synthetase (ASS) in the liver cytosol. (wikipedia.org)
  • Quantitative PCR was utilized to determine liver GS, CPS, OCT, ARG, argininosuccinate synthetase (ASS) and argininosuccinate lyase (ASL) mRNA expression. (biologists.org)
  • Assay of argininosuccinate lyase may be determined in cultured fibroblasts (skin) or tissue (liver) biopsies and erythrocytes. (renalandurologynews.com)
  • Argininosuccinate (ASA) lyase deficiency results in defective cleavage of ASA. (medscape.com)
  • The specific role of the argininosuccinate lyase enzyme is to start the reaction in which the amino acid arginine, a building block of proteins, is produced from argininosuccinate, the molecule that carries the waste nitrogen collected earlier in the urea cycle. (medlineplus.gov)
  • Tumor necrosis factor-reduces argininosuccinate symthase expression and nitric oxide production in aorticendothelial cells. (thefreedictionary.com)
  • Patients with argininosuccinate lyase deficiency (ASLD) will be randomly assigned to receive either a nitric oxide dietary supplement or placebo for 24 weeks, and then crossed-over to receive the other treatment for 24 weeks. (clinicaltrials.gov)
  • ARLY_ACISJ Argininosuccinate lyase OS=Acidovorax sp. (uniprot.org)
  • One colony was recovered which contained the YEp13 plasmid bearing a large insert complementing the argininosuccinate lyase (ASL) mutation. (springer.com)
  • Argininosuccinate lyase (ASL) deficiency: mutation analysis in 27 patients and a completed structure of the human ASL gene. (medlineplus.gov)
  • Although intellectual impairment is the rule, even among patients who receive excellent and timely treatment, some patients with ASA lyase deficiency reportedly develop normally. (medscape.com)
  • Argininosuccinate lyase deficiency: mutational spectrum in Italian patients and identification of a novel ASL pseudogene. (semanticscholar.org)
  • 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)
  • Immunofluorescence analysis of paraformaldehyde-fixed HeLa, using Argininosuccinate Lyase(GTX113629) antibody at 1:200 dilution. (genetex.com)
  • Argininosuccinate neurotoxicity and prevention by creatine in argininosuccinate lyase deficiency: an in vitro study in rat 3D organotypic brain cell cultures. (unibe.ch)
  • each is referred to by the initials of the missing enzyme: CPS1 (Carbamoyl Phosphate Synthetase), NAGS (N-Acetylglutamate Synthetase), OTC Deficiency (Ornithine Transcarbamylase), AS (Argininosuccinic Acid Synthetase (Citrullinemia)), ASL (Argininosuccinate Lyase (Argininosuccinic Aciduria)), and AG (Arginase). (lucknownewsflash.in)
  • Mutations in the ARGININOSUCCINATE LYASE gene cause the disorder. (selfdecode.com)
  • Abstract Plasmid DNA carrying either the nitrate reductase (NR) gene or the argininosuccinate lyase gene as selectable markers and the corresponding Chlamydomonas reinhardtii mutants as recipient strains have been used to isolate regulatory mutants for nitrate assimilation by insertional. (nationallizenzen.de)