Adenylosuccinate Lyase: An enzyme that, in the course of purine ribonucleotide biosynthesis, catalyzes the conversion of 5'-phosphoribosyl-4-(N-succinocarboxamide)-5-aminoimidazole to 5'-phosphoribosyl-4-carboxamide-5-aminoimidazole and the conversion of adenylosuccinic acid to AMP. EC 4.3.2.2.Adenylosuccinate Synthase: A carbon-nitrogen ligase. During purine ribonucleotide biosynthesis, this enzyme catalyzes the synthesis of adenylosuccinate from GTP; IMP; and aspartate with the formation of orthophosphate and GDP. EC 6.3.4.4.Purine-Pyrimidine Metabolism, Inborn ErrorsLyases: 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.Aminoimidazole Carboxamide: An imidazole derivative which is a metabolite of the antineoplastic agents BIC and DIC. By itself, or as the ribonucleotide, it is used as a condensation agent in the preparation of nucleosides and nucleotides. Compounded with orotic acid, it is used to treat liver diseases.Purines: A series of heterocyclic compounds that are variously substituted in nature and are known also as purine bases. They include ADENINE and GUANINE, constituents of nucleic acids, as well as many alkaloids such as CAFFEINE and THEOPHYLLINE. Uric acid is the metabolic end product of purine metabolism.Inosine Monophosphate: Inosine 5'-Monophosphate. A purine nucleotide which has hypoxanthine as the base and one phosphate group esterified to the sugar moiety.Ribonucleotides: Nucleotides in which the purine or pyrimidine base is combined with ribose. (Dorland, 28th ed)Adenosine Monophosphate: Adenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position.Thermotoga maritima: A rod-shaped bacterium surrounded by a sheath-like structure which protrudes balloon-like beyond the ends of the cell. It is thermophilic, with growth occurring at temperatures as high as 90 degrees C. It is isolated from geothermally heated marine sediments or hot springs. (From Bergey's Manual of Determinative Bacteriology, 9th ed)Bacillus subtilis: A species of gram-positive bacteria that is a common soil and water saprophyte.Mutation, Missense: A mutation in which a codon is mutated to one directing the incorporation of a different amino acid. This substitution may result in an inactive or unstable product. (From A Dictionary of Genetics, King & Stansfield, 5th ed)Molecular Sequence Data: 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.Kinetics: The rate dynamics in chemical or physical systems.Amino Acid Sequence: 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.Dictionaries, MedicalDictionaries as Topic: Lists of words, usually in alphabetical order, giving information about form, pronunciation, etymology, grammar, and meaning.Pyrobaculum: A genus of rod-shaped, almost rectangular ARCHAEA, in the family THERMOPROTEACEAE. Organisms are facultatively aerobic or strictly anaerobic, grow on various organic substrates, and are found in continental solfataras.Thermoproteaceae: A family of THERMOPROTEALES consisting of variable length rigid rods without septa. They grow either chemolithoautotrophically or by sulfur respiration. The four genera are: PYROBACULUM; THERMOPROTEUS; Caldivirga; and Thermocladium. (From Bergey's Manual of Systematic Bacteriology, 2d ed)Argininosuccinic Aciduria: 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.Love: Affection; in psychiatry commonly refers to pleasure, particularly as it applies to gratifying experiences between individuals.Steroid 17-alpha-Hydroxylase: A microsomal cytochrome P450 enzyme that catalyzes the 17-alpha-hydroxylation of progesterone or pregnenolone and subsequent cleavage of the residual two carbons at C17 in the presence of molecular oxygen and NADPH-FERRIHEMOPROTEIN REDUCTASE. This enzyme, encoded by CYP17 gene, generates precursors for glucocorticoid, androgen, and estrogen synthesis. Defects in CYP17 gene cause congenital adrenal hyperplasia (ADRENAL HYPERPLASIA, CONGENITAL) and abnormal sexual differentiation.Alleles: Variant forms of the same gene, occupying the same locus on homologous CHROMOSOMES, and governing the variants in production of the same gene product.Internet: 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.Information Storage and Retrieval: Organized activities related to the storage, location, search, and retrieval of information.User-Computer Interface: The portion of an interactive computer program that issues messages to and receives commands from a user.Software: Sequential operating programs and data which instruct the functioning of a digital computer.Algorithms: A procedure consisting of a sequence of algebraic formulas and/or logical steps to calculate or determine a given task.United StatesDual-Specificity Phosphatases: A sub-class of protein tyrosine phosphatases that contain an additional phosphatase activity which cleaves phosphate ester bonds on SERINE or THREONINE residues that are located on the same protein.MicroRNAs: Small double-stranded, non-protein coding RNAs, 21-25 nucleotides in length generated from single-stranded microRNA gene transcripts by the same RIBONUCLEASE III, Dicer, that produces small interfering RNAs (RNA, SMALL INTERFERING). They become part of the RNA-INDUCED SILENCING COMPLEX and repress the translation (TRANSLATION, GENETIC) of target RNA by binding to homologous 3'UTR region as an imperfect match. The small temporal RNAs (stRNAs), let-7 and lin-4, from C. elegans, are the first 2 miRNAs discovered, and are from a class of miRNAs involved in developmental timing.Streptomyces griseus: An actinomycete from which the antibiotics STREPTOMYCIN, grisein, and CANDICIDIN are obtained.3' Untranslated Regions: The sequence at the 3' end of messenger RNA that does not code for product. This region contains transcription and translation regulating sequences.Protein Tyrosine Phosphatases: An enzyme group that specifically dephosphorylates phosphotyrosyl residues in selected proteins. Together with PROTEIN-TYROSINE KINASE, it regulates tyrosine phosphorylation and dephosphorylation in cellular signal transduction and may play a role in cell growth control and carcinogenesis.Insectivora: An order of insect eating MAMMALS including MOLES; SHREWS; HEDGEHOGS and tenrecs.Cyclin-Dependent Kinases: Protein kinases that control cell cycle progression in all eukaryotes and require physical association with CYCLINS to achieve full enzymatic activity. Cyclin-dependent kinases are regulated by phosphorylation and dephosphorylation events.MedlinePlus: 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.Health Records, Personal: Longitudinal patient-maintained records of individual health history and tools that allow individual control of access.SulfonesOnline Systems: Systems where the input data enter the computer directly from the point of origin (usually a terminal or workstation) and/or in which output data are transmitted directly to that terminal point of origin. (Sippl, Computer Dictionary, 4th ed)PiperazinesPhosphodiesterase Inhibitors: Compounds which inhibit or antagonize the biosynthesis or actions of phosphodiesterases.Phosphodiesterase 5 Inhibitors: Compounds that specifically inhibit PHOSPHODIESTERASE 5.Erectile Dysfunction: The inability in the male to have a PENILE ERECTION due to psychological or organ dysfunction.Adenosine: A nucleoside that is composed of ADENINE and D-RIBOSE. Adenosine or adenosine derivatives play many important biological roles in addition to being components of DNA and RNA. Adenosine itself is a neurotransmitter.Receptor, Adenosine A2A: A subclass of adenosine A2 receptors found in LEUKOCYTES, the SPLEEN, the THYMUS and a variety of other tissues. It is generally considered to be a receptor for ADENOSINE that couples to the GS, STIMULATORY G-PROTEIN.Receptor, Adenosine A3: A subtype of ADENOSINE RECEPTOR that is found expressed in a variety of locations including the BRAIN and endocrine tissues. The receptor is generally considered to be coupled to the GI, INHIBITORY G-PROTEIN which causes down regulation of CYCLIC AMP.Receptor, Adenosine A1: A subtype of ADENOSINE RECEPTOR that is found expressed in a variety of tissues including the BRAIN and DORSAL HORN NEURONS. The receptor is generally considered to be coupled to the GI, INHIBITORY G-PROTEIN which causes down regulation of CYCLIC AMP.Adenosine Deaminase: An enzyme that catalyzes the hydrolysis of ADENOSINE to INOSINE with the elimination of AMMONIA.Receptors, Adenosine A2: A subclass of ADENOSINE RECEPTORS that are generally considered to be coupled to the GS, STIMULATORY G-PROTEIN which causes up regulation of CYCLIC AMP.Ribose: A pentose active in biological systems usually in its D-form.

Capillary electrophoresis for detection of inherited disorders of purine and pyrimidine metabolism. (1/51)

BACKGROUND: Measurement of purine and pyrimidine metabolites presents complex problems for separations currently performed by HPLC and thin-layer chromatography in clinical practice. We developed a novel capillary electrophoresis method for this purpose. METHODS: Separations were performed in 60 mmol/L borate-2-amino-2-methyl-1-propanol-80 mmol/L sodium dodecyl sulfate (pH 9.6) at 35 degrees C. RESULTS: The conditions reported allowed separation of all diagnostic metabolites from major urinary constituents in an analysis time of 3 min and with a separation efficiency of 220 000 theoretical plates/m. The clinically important metabolites were detectable at concentrations of 0.85-4.28 micromol/L. The method was linear over the range 5-500 micromol/L (r >0.99). The within-run and intra- and interday imprecision (CV) was <5%. Characteristic abnormalities were detected in the electropherograms of urine samples from patients with purine and pyrimidine enzyme deficiencies. We provide the electrophoretic and spectral characteristics of many intermediates in purine and pyrimidine metabolism and describe common artifacts from medication and ultraviolet-absorbing compounds. CONCLUSION: Capillary electrophoresis is a valuable screening tool in the detection of inborn errors of purine and pyrimidine metabolism.  (+info)

The structure of adenylosuccinate lyase, an enzyme with dual activity in the de novo purine biosynthetic pathway. (2/51)

BACKGROUND: Adenylosuccinate lyase is an enzyme that plays a critical role in both cellular replication and metabolism via its action in the de novo purine biosynthetic pathway. Adenylosuccinate lyase is the only enzyme in this pathway to catalyze two separate reactions, enabling it to participate in the addition of a nitrogen at two different positions in adenosine monophosphate. Both reactions catalyzed by adenylosuccinate lyase involve the beta-elimination of fumarate. Enzymes that catalyze this type of reaction belong to a superfamily, the members of which are homotetramers. Because adenylosuccinate lyase plays an integral part in maintaining proper cellular metabolism, mutations in the human enzyme can have severe clinical consequences, including mental retardation with autistic features. RESULTS: The 1.8 A crystal structure of adenylosuccinate lyase from Thermotoga maritima has been determined by multiwavelength anomalous dispersion using the selenomethionine-substituted enzyme. The fold of the monomer is reminiscent of other members of the beta-elimination superfamily. However, its active tetrameric form exhibits striking differences in active-site architecture and cleft size. CONCLUSIONS: This first structure of an adenylosuccinate lyase reveals that, along with the catalytic base (His141) and the catalytic acid (His68), Gln212 and Asn270 might play a vital role in catalysis by properly orienting the succinyl moiety of the substrates. We propose a model for the dual activity of adenylosuccinate lyase: a single 180 degrees bond rotation must occur in the substrate between the first and second enzymatic reactions. Modeling of the pathogenic human S413P mutation indicates that the mutation destabilizes the enzyme by disrupting the C-terminal extension.  (+info)

Succinylpurinemic autism: increased sensitivity of defective adenylosuccinate lyase towards 4-hydroxy-2-nonenal. (3/51)

We studied the effect of trans-4-hydroxy-2-nonenal on the wild-type human adenylosuccinate lyase and on the enzyme from a patient compound-heterozygous for two missense mutations (P75A/D397Y; McKusick 103050.0003/103050.0004). Both the enzymes were inhibited by 10-50 microM trans-4-hydroxy-2-nonenal in a concentration-dependent manner by means of a mixed-type co-operative mechanism. A significantly stronger inhibition was noticed in the presence of the defective enzyme. Nonanal and trans-2,3-nonenal inhibited the enzymes to a less extent and at about 10-times higher concentrations. Hydroxylamine reversed the inhibition by trans-4-hydroxy-2-nonenal, trans-2,3-nonenal or nonanal in the case of the wild-type enzyme, but it was ineffective to reverse the inhibition by trans-4-hydroxy-2-nonenal on the defective enzyme. Dithiothreitol slightly decreased the inhibition exerted by trans-4-hydroxy-2-nonenal on both the wild-type and the defective adenylosuccinate lyase, while it did not produce practically any change in the presence of trans-2,3-nonenal or nonanal.  (+info)

Human adenylosuccinate lyase (ADSL), cloning and characterization of full-length cDNA and its isoform, gene structure and molecular basis for ADSL deficiency in six patients. (4/51)

Adenylosuccinate lyase (ADSL) is a bifunctional enzyme acting in de novo purine synthesis and purine nucleotide recycling. ADSL deficiency is a selectively neuronopathic disorder with psychomotor retardation and epilepsy as leading traits. Both dephosphorylated enzyme substrates, succinylaminoimidazole-carboxamide riboside (SAICAr) and succinyladenosine (S-Ado), accumulate in the cerebrospinal fluid (CSF) of affected individuals with S-Ado/SAICAr concentration ratios proportional to the phenotype severity. We studied the disorder at various levels in a group of six patients with ADSL deficiency. We identified the complete ADSL cDNA and its alternatively spliced isoform resulting from exon 12 skipping. Both mRNA isoforms were expressed in all the tissues studied with the non-spliced form 10-fold more abundant. Both cDNAs were expressed in Escherichia coli and functionally characterized at the protein level. The results showed only the unspliced ADSL to be active. The gene consists of 13 exons spanning 23 kb. The promotor region shows typical features of the housekeeping gene. Eight mutations were identified in a group of six patients. The expression studies of the mutant proteins carried out in an attempt to study genotype-phenotype correlation showed that the level of residual enzyme activity correlates with the severity of the clinical phenotype. All the mutant enzymes studied in vitro displayed a proportional decrease in activity against both of their substrates. However, this was not concordant with strikingly different concentration ratios in the CSF of individual patients. This suggests either different in vivo enzyme activities against each of the substrates and/or their different turnover across the CSF-blood barrier, which may be decisive in determining disease severity.  (+info)

Clinical, biochemical and molecular genetic correlations in adenylosuccinate lyase deficiency. (5/51)

Adenylosuccinate lyase (ADSL) deficiency (MIM 103050) is an autosomal recessive inborn error of purine synthesis characterized by the accumulation in body fluids of succinylaminoimidazolecarboxamide (SAICA) riboside and succinyladenosine (S-Ado), the dephosphorylated derivatives of the two substrates of the enzyme. Because ADSL-deficient patients display widely variable degrees of psychomotor retardation, we have expressed eight mutated ADSL enzymes as thioredoxin fusions and compared their properties with the clinical and biochemical characteristics of 10 patients. Three expressed mutated ADSL enzymes (M26L, R426H and T450S) were thermolabile, four (A2V, R141W, R303C and S395R) were thermostable and one (del206-218), was inactive. Thermolabile mutations decreased activities with SAICA ribotide (SAICAR) and adenylosuccinate (S-AMP) in parallel, or more with SAICAR than with S-AMP. Patients homozygous for one of these mutations, R426H, displayed similarly decreased ADSL activities in their fibroblasts, S-Ado:SAICA riboside ratios of approximately 1 in their cerebrospinal fluid and were profoundly retarded. With the exception of A2V, thermostable mutations decreased activity with S-AMP to a much more marked extent than with SAICAR. Two unrelated patients homozygous for one of the thermostable mutations, R303C, also displayed a much more marked decrease in the activity of fibroblast ADSL with S-AMP than with SAICAR, had S-Ado:SAICA riboside ratios between 3 and 4 in their cerebrospinal fluid and were mildly retarded. These results suggest that, in some cases, the genetic lesion of ADSL determines the ratio of its activities with S-AMP versus SAICAR, which in turn defines the S-Ado:SAICA riboside ratio and the patients' mental status.  (+info)

Mutation of a nuclear respiratory factor 2 binding site in the 5' untranslated region of the ADSL gene in three patients with adenylosuccinate lyase deficiency. (6/51)

Adenylosuccinate lyase (ADSL; also called "adenylosuccinase") catalyzes two steps in the synthesis of purine nucleotides: (1) the conversion of succinylaminoimidazolecarboxamide ribotide into aminoimidazolecarboxamide ribotide and (2) the conversion of adenylosuccinate into adenosine monophosphate. ADSL deficiency, a recessively inherited disorder, causes variable-but most often severe-mental retardation, frequently accompanied by epilepsy and/or autism. It is characterized by the accumulation, in body fluids, of succinylaminoimidazolecarboxamide riboside and succinyladenosine, the dephosphorylated derivatives of the two substrates of the enzyme. Analysis of the ADSL gene of three unrelated patients with ADSL deficiency, in whom one of the ADSL alleles displayed a normal coding sequence, revealed a -49T-->C mutation in the 5' untranslated region of this allele. Measurements of the amount of mRNA transcribed from the latter allele showed that it was reduced to approximately 33% of that transcribed from the alleles mutated in their coding sequence. Further investigations showed that the -49T-->C mutation provokes a reduction to 25% of wild-type control of promoter function, as evaluated by luciferase activity and mRNA level in transfection experiments. The mutation also affects the binding of nuclear respiratory factor 2 (NRF-2), a known activator of transcription, as assessed by gel-shift studies. Our findings indicate that a mutation of a regulatory region of the ADSL gene might be an unusually frequent cause of ADSL deficiency, and they suggest a role for NRF-2 in the gene regulation of the purine biosynthetic pathway.  (+info)

Splicing graphs and EST assembly problem. (7/51)

MOTIVATION: The traditional approach to annotate alternative splicing is to investigate every splicing variant of the gene in a case-by-case fashion. This approach, while useful, has some serious shortcomings. Recent studies indicate that alternative splicing is more frequent than previously thought and some genes may produce tens of thousands of different transcripts. A list of alternatively spliced variants for such genes would be difficult to build and hard to analyse. Moreover, such a list does not show the relationships between different transcripts and does not show the overall structure of all transcripts. A better approach would be to represent all splicing variants for a given gene in a way that captures the relationships between different splicing variants. RESULTS: We introduce the notion of the splicing graph that is a natural and convenient representation of all splicing variants. The key difference with the existing approaches is that we abandon the linear (sequence) representation of each transcript and replace it with a graph representation where each transcript corresponds to a path in the graph. We further design an algorithm to assemble EST reads into the splicing graph rather than assembling them into each splicing variant in a case-by-case fashion.  (+info)

The characterization of mutant Bacillus subtilis adenylosuccinate lyases corresponding to severe human adenylosuccinate lyase deficiencies. (8/51)

Adenylosuccinate lyase is a homotetramer that catalyzes two discrete reactions in the de novo synthesis of purines: the cleavage of adenylosuccinate and succinylaminoimidazole carboxamide ribotide (SAICAR). Several point mutations in the gene encoding the enzyme have been implicated in human disease. Bacillus subtilis adenylosuccinate lyase was used as a model system in which mutations were constructed corresponding to those mutations associated with severe human adenylosuccinate lyase deficiency. Site-directed mutagenesis was utilized to construct amino acid substitutions in B. subtilis adenylosuccinate lyase; Met(10), Ile(123), and Thr(367) were replaced by Leu, Trp, and Arg, respectively, and the altered enzymes were expressed in Escherichia coli. These purified enzymes containing amino acid substitutions were found to have substantial catalytic activity and exhibit relatively small changes in their kinetic parameters. The major deviations from the wild-type-like behavior were observed upon biophysical characterization. All of these enzymes with amino acid replacements are associated with marked thermal instability. I123W adenylosuccinate lyase exhibits notable changes in the circular dichroism spectra, and a native gel electrophoresis pattern indicative of some protein aggregation. T367R also exhibits alterations at the quarternary level, as reflected in native gel electrophoresis. Experimental results, combined with homology modeling, suggest that the altered enzymes are primarily structurally impaired. The enzyme instability was found to be lessened by subunit complementation with the wild-type enzyme, under mild conditions; these studies may have implications for the in vivo behavior of adenylosuccinate lyase in heterozygous patients. Residues Met(10), Ile(123), and Thr(367) appear to be located in regions of the enzyme important for maintaining the structural integrity required for a stable, functional enzyme.  (+info)

2VD6: Human adenylosuccinate lyase in complex with its substrate N6-(1,2- Dicarboxyethyl)-AMP, and its products AMP and fumarate.
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Adenylosuccinate lyase is an enzyme of fumarase superfamily that participates in the purine biosynthetic pathway, catalysing the nonhydrolytic cleavage of succinyl groups from SAICA ribotide and adenylosuccinate. Enzyme defects are associated with a
Definition of adenylosuccinate lyase. Provided by Stedmans medical dictionary and Drugs.com. Includes medical terms and definitions.
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 ...
1F1O: The crystal structure of adenylosuccinate lyase from Pyrobaculum aerophilum reveals an intracellular protein with three disulfide bonds.
Adenylosuccinate Lyase; Catalyzes Two Steps In The de Novo Purine Nucleotide Biosynthetic Pathway; Expression Is Repressed By Adenine And Activated By Bas1p And Pho2p; Mutations In Human Ortholog ADSL Cause Adenylosuccinase Deficiency
Adenylosuccinate Lyase; Catalyzes Two Steps In The de Novo Purine Nucleotide Biosynthetic Pathway; Expression Is Repressed By Adenine And Activated By Bas1p And Pho2p; Mutations In Human Ortholog ADSL Cause Adenylosuccinase Deficiency
Adenylosuccinate lyase (ASL) catalyzes 2 reactions in the de novo synthesis of purine nucleotides. In humans, point mutations of this enzyme are associated with mental retardation, autism, and muscle wasting. The sequence of B. subtilis ASL is 27% identical and 17% similar to the human enzyme. We have cloned, expressed, and purified B. subtilis ASL; the crystal structure of a 73% homologous bacterial enzyme is known (PDB #lc3u). We separately mutated 2 amino acids in the B. subtilis ASL (M10L & N276C) equivalent to the mutations identified in human ASL deficiency (M26L & R303C). The purified M10L and N276C possess specific activities of 0.86 and 0.38 units/mg, respectively, as compared to 1.56 units/mg for wild type (WT) enzyme. Both enzymes are similar to WT in conformation and oligomeric state as indicated by circular dichroism and light scattering. The Km for adenylosuccinate (SAMP) is not appreciably different between M1OL (5.6 uM) and WT (4.9 uM); and the pH dependence of Vmax is also ...
Banerjee, Sanchari and Agrawal, Monika J and Mishra, Diptimayee and Sharan, Siddharth and Balaram, Hemalatha and Savithri, Handanhal S and Murthy, Mathur RN (2014) Structural and kinetic studies on adenylosuccinate lyase from Mycobacterium smegmatis and Mycobacterium tuberculosis provide new insights on the catalytic residues of the enzyme. In: FEBS JOURNAL, 281 (6). pp. 1642-1658. Chittori, Sagar and Simanshu, Dhirendra Kumar and Banerjee, Sanchari and Murthy, Ambika Mosale Venkatesh and Mathivanan, Subashini and Savithri, Handanahal Subbarao and Murthy, Mathur Ramabhadrashastry Narasimha (2013) Mechanistic features of Salmonella typhimurium propionate kinase (TdcD): Insights from kinetic and crystallographic studies. In: BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS, 1834 (10). pp. 2036-2044. ...
Looking for online definition of SAICAR or what SAICAR stands for? SAICAR is listed in the Worlds largest and most authoritative dictionary database of abbreviations and acronyms
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Our road infrastructure has an upto 70mph limit.. My rather old landrover will only do about 50mph, so perhaps I should only pay 71% of the Road Tax for my vehicle.. There are several national speed limit roads near where I live. I defy anyone to drive down them at 60mph, even though that is the therectical limit.. When I bought my latest vehicle, I checked the official fuel consumption figures. Apparently I can get upto 52mpg. Like that will ever happen! Even if I drive like a nun.. These days, most ISPs package their products with download limits, rather than speed.. The upto speed is only the maximum of the technology they are using. They could say they use ADSL, ADSL2 & let the customer work out what speed they are likely to get.. Anybody know who to contact to get my Road Tax refunded?. ...
This gene encodes a member of the adenylosuccinate synthase family of proteins. The encoded muscle-specific enzyme plays a role in the purine nucleotide cycle by catalyzing the first step in the conversion of inosine monophosphate (IMP) to adenosine monophosphate (AMP). Mutations in this gene may cause adolescent onset distal myopathy. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Feb 2016 ...
Plays an important role in the de novo pathway and in the salvage pathway of purine nucleotide biosynthesis. Catalyzes the first committed step in the biosynthesis of AMP from IMP.
Adenylosuccinate synthetase; Plays an important role in the de novo pathway of purine nucleotide biosynthesis. Catalyzes the first committed step in the biosynthesis of AMP from IMP; Belongs to the adenylosuccinate synthetase family (333 aa ...
K. Eaazhisai, R. Jayalakshmi, P. Gayathri, R. P. Anand, K. Sumathy, H. Balaram and M. R. N. Murthy, Crystal structure of fully-ligated adenylosuccinate synthetase from Plasmodium falciparum, Journal of Molecular Biology 335, 1251 - 1264 (2004 ...
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Wholesale supplier estimates the reported service issue will be investigated on or before 27/06/2018 7:00PM. Please ensure suitable access to main distribution frame or the network boundary point is available. Usually when the technician is arranged as a commitment, they dont need to have access inside the premises. However we may need to wait without having further changes or troubleshooting until the technician visit is completed ...
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Hi all, Im currently having problems with ny network at home. Im currently using an old (about 5 years old) Belkin router, ADSL 2+ which is connected...
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On June my PC was invaded by a virus (probably Rombertik or similar). I could not eliminate it.On August I moved of house and address. I hired a new ADSL
While the package does not explicitly say that up to speeds are to be outlawed, a statement by Neelie Kroes suggests that this may be the intent "no more "up to 30 Megabit" deals that in practice just offer 3". The UK already has broadband speeds code of practice but it is voluntary and this new set of rules are defining rights enshrined in law. So what actual data speeds really means is important, but for now that is anyones guess, the existing UK code of practice may be enough, but be compulsory rather than voluntary. Certainly the UK moving to a gaining provider led system for migrations in 2015 ticks the switching box.. We know many who will applaud the end to up to speeds since it will expose real performance of ADSL and VDSL2 based services, but it will also make life harder for the various fixed speed connection services such as cable and FTTH/FTTP/FTTB, since contention and shared access on the backhaul networks is the sole reason that mortals can afford broadband connections at ...
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17α-hydroxylase/17,20-lyase deficiency is caused by mutations in the CYP17A1 gene. The protein produced from this gene is involved in the formation of steroid hormones. This group of hormones includes sex hormones such as testosterone and estrogen, which are needed for normal sexual development and reproduction; mineralocorticoids, which help regulate the bodys salt and water balance; and glucocorticoids, which are involved in maintaining blood sugar levels and regulating the bodys response to stress.. Steroid hormones are produced through a series of chemical reactions. The CYP17A1 enzyme performs two important reactions in this process. The enzyme has 17 alpha(α)-hydroxylase activity, which is important for production of glucocorticoids and sex hormones. CYP17A1 also has 17,20-lyase activity, which is integral to the production of sex hormones.. 17α-hydroxylase/17,20-lyase deficiency results from a shortage (deficiency) of both enzyme activities. The amount of remaining enzyme activity ...
ID D1YV79_METPS Unreviewed; 337 AA. AC D1YV79; DT 09-FEB-2010, integrated into UniProtKB/TrEMBL. DT 09-FEB-2010, sequence version 1. DT 11-DEC-2019, entry version 67. DE RecName: Full=Adenylosuccinate synthetase {ECO:0000256,HAMAP-Rule:MF_00011, ECO:0000256,RuleBase:RU000520}; DE Short=AMPSase {ECO:0000256,HAMAP-Rule:MF_00011}; DE Short=AdSS {ECO:0000256,HAMAP-Rule:MF_00011}; DE EC=6.3.4.4 {ECO:0000256,HAMAP-Rule:MF_00011, ECO:0000256,RuleBase:RU000520}; DE AltName: Full=IMP--aspartate ligase {ECO:0000256,HAMAP-Rule:MF_00011}; GN Name=purA {ECO:0000256,HAMAP-Rule:MF_00011, GN ECO:0000313,EMBL:BAI60351.1}; GN OrderedLocusNames=MCP_0279 {ECO:0000313,EMBL:BAI60351.1}; OS Methanocella paludicola (strain DSM 17711 / JCM 13418 / NBRC 101707 / OS SANAE). OC Archaea; Euryarchaeota; Stenosarchaea group; Methanomicrobia; OC Methanocellales; Methanocellaceae; Methanocella. OX NCBI_TaxID=304371 {ECO:0000313,EMBL:BAI60351.1, ECO:0000313,Proteomes:UP000001882}; RN [1] {ECO:0000313,Proteomes:UP000001882} RP ...
SWISS-MODEL Repository entry for A8Q0E3 (PURA_MALGO), Adenylosuccinate synthetase. Malassezia globosa (strain ATCC MYA-4612 / CBS 7966)(Dandruff-associated fungus)
Rabbit polyclonal antibody raised against a full-length human ASL protein. ASL (NP_000039.2, 1 a.a. ~ 464 a.a) full-length human protein. (H00000435-D01P) - Products - Abnova
An improved discrete multi-tone transmission scheme is describe that contemplates encoding digital data and modulating the encoded data onto a discrete multi-tone signal having a total bandwidth of at least 1.6 MHz. The modulation system is capable of dynamically updating the subcarriers used and the amount of data transmitted on each subcarrier during transmission in order to accommodate real time changes in the line quality over particular subcarriers. In one preferred embodiment of the invention the multi-tone encoding and modulation is done in substantial compliance with the ATIS North American Asymmetric Digital Subscriber Lines standard although a total of 512 subchannels each having a bandwidth of approximately 4.3125 kHz are used. In this system, the subchannels that occur at frequencies above those set forth in the standard are treated similarly to those within the standard range in terms of subcarrier selection criteria. This embodiment contemplates the use of a discrete multi-tone signal
Which should give you the revision number of your modem. If your modem is revision 0 or 2 you will need the KQD6_3.012 file. If your modem is revision 4, then you will require the ZZZL_3.012 file. Both of these files can be located in this tarball here. You will, later, need the firmware-extractor too, which can be found here. Finally you should check this page to find out the VPI/VCI numbers for your country or ISP. Now you have everything, save a copy of this guide too (in text helps), and copy everything to a USB flash drive, floppy disk (if it fits), or a USB external hard drive, so you can use them while offline. NOTE: Unzip isnt supported in a basic Arch Linux installation, you should unzip any .zip files using another computer or operating system first. ...
RSAWEB Connecting Tomorrow.. In this increasingly digital age it is imperative that businesses and individuals harness the power of the Internet in order to stay ahead. RSAWEB makes the Internet work for you by connecting people, places, platforms and applications to make your life easier, faster and ultimately better.. RSAWEB offers the best in connectivity and hosting products for both businesses and individuals. Whether it is ADSL , Fibre, Mobile Data or Cloud solutions that you are looking for, RSAWEB has a solution to suit your needs and budget.. Since 2001, RSAWEB has grown rapidly, gaining extensive industry experience and expertise while continuously improving their products and pushing boundaries. With great commitment to customer service excellence and in-depth technical understanding, RSAWEB retains partnerships with some of SAs leading companies.. "We bridge the gap between IT innovation and everyday life - and the RSAWEB team takes pride in doing this every day." - RSAWEB Director, ...
I want to make the leap to linux (specifically Redhat 7.2) but Im pretty sure my modem isnt gonna work with it. Can anyone recommend a good modem that I should get? Is an external modem a good bet? Also what about cable/adsl modems, as I think pretty soon in the future Im going to go for broadband, what are peoples experience with getting these to work? thanks a lot, hush
Alle 16:39 di sabato 8 dicembre 2007, Francesco Groccia ha scritto: , Leonardo Canducci ,[email protected], writes: , , Il giorno sab, 08/12/2007 alle 12.00 +0100, Francesco Groccia ha , , , , scritto: , , , se ti può essere utile, ho preso un router adsl a meno di 25 , , , euro, firewall integrato, 4 porte ethernet, configurabile via , , , browser, supporta ADSL2 e 2+ (quindi anche le linee veloci di , , , ultima generazione) , , , , marca? modello? , , Sweex annex fiko, ma a meno di 44€ non lho trovato. a 25 dove? -- Franco Alle prossime elezioni voterò Bakunin, che purtroppo non è in lista. Luciano Bianciardi, 8 marzo 1971 ...
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I couldnt get away from Sky soon enough. Was with them on ADSL for a while, and had constant problems with the crappy router they provided. There was a bug in its firmware that meant that connected devices were never displayed properly. I either got a list of the same device connected repeatedly, or, an incomprehensible list of devices in what looked like wingdings. I complained to Sky, who acknowledged that they were aware of the problem, and that Dlink had released a firmware update several years earlier. Sky refused to release that for their customers and also refused to give me my username and password, to allow me to use a decent router.. When fibre became available Sky offered up to 40Mbps, BT offered up to 70Mbps for the same price. We get well over the 40 offered by Sky, usually hitting around 60.. The process of leaving was painful, with quite probably the rudest call centre handler in history, who was shouting and refusing to listen, intent on going through her script of bribes to ...
Well, note to self: dont try doing tech support when late at night. That ADSL modem router WiFi firewall box thingy that apparently wouldnt work anymore? It does work. It did come up on its default address of 192.168.1.1, but there was a reason Id failed to be able to connect to it. Now, Im…
F-, fhuA2::IS2, lacY1, tsx-70, glnX44(AS), gal-6, λ-, purB51, hemA30, trpC45, pabB5, his-68, rfbC1, tyrA2, galP63, rpsL125(strR), malT1(λR), xyl-7, mtlA2, thiE1, pyr-65 ...
Discover Lifes page about the biology, natural history, ecology, identification and distribution of Augochlora pura - -- Discover Life
The effect of 3-mercaptopicolinate, an inhibitor of phosphoenolpyruvate carboxykinase [GTP:oxaloacetate carboxy-lyase (transphosphorylating), EC 4.1.1.32], was tested on NH3 formation via the purine nucleotide cycle and glutamate dehydrogenase (EC 1.4.1.2). NH3 excretion in rats increased 70-fold after 48 h of NH4Cl feeding, from 12.2 +/- 4.5 to 862 +/- 190 mumol/mg of creatinine. At 4 h after a single intraperitoneal injection of 3-mercaptopicolinate into NH4Cl-fed rats, NH3 excretion was inhibited by 93%. Kidneys of NH4Cl-fed plus 3-mercaptopicolinate-treated rats, compared with those of NH4Cl-fed rats, showed a 3.5-fold increase in the content of IMP, 5-fold increase in adenylosuccinate, 4-fold increase in aspartate, and a 30% increase in AMP. 3-Mercaptopicolinate completely inhibited NH3 and glucose formation from glutamate in tubules from acidotic rats and NH3 formation from aspartate in kidney perfusion experiments. When transamination in tubules was prevented by ...
Our lab studies hypoxanthine guanine phosphoribosyltransferase and adenylosuccinate synthetase, enzymes involved in purine salvage in the malarial parasite, Plasmodium falciparum. We have also been studying enzymes involved in hemoglobin degradation and glycolysis. Enzymes of these essential pathways are of interest as targets for antimalarial chemotherapy. Protein engineering/ mutagenesis, spectroscopy and X- Ray crystallographic techniques are used to probe the structure, function and dynamics of these enzymes. Other aspects studied include substrate specificity,catalytic and kinetic mechanisms, and protein stability. Insights from these studies would aid the development of new antimalarials.. ...
Easy Driver Pro performs a complete scan of your all of the devices in or attached to your computer. All rights reserved. It can determine which D-Link DSL-200 Modem Drivers For Windows 8 are either missing, corrupt, or have become obsolete. If you have found our site useful, we will appreciate it if you tell your friends about us in social networks. D-Link DSL-200 USB ADSL Modem(WAN):The D-Link DSL-200 is an external plug and play ADSL USB modem The DSL-200 allows a Windows based PC to connect via Glite or Gdmt ADSL to Easy Driver Pro free scan will find all the outdated drivers on your PC. Auto-Tune Evo VST 6.0.9.2 Corrects vocals or solo instruments. http://atlantecsoftware.com/dslw200-modem/d-link-dsl-200-drivers-download.html Easy Driver Pro will scan your computer for missing, corrupt, and outdated Drivers. Learn more or ✐ Close Software Free Download , Soft32.com Home Windows Mac Mobile Blog Search You can skip this in seconds Click here to continue Download Now! Please read the ...
Thank you for your interest in spreading the word about Biochemical Society Transactions.. NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.. ...
Pediatr Neurol. 2007 Jul;37(1):47-50. 3-Hydroxy-3-methylglutaryl coenzyme a lyase deficiency with reversible white matter changes after treatment. Zafeiriou DI, Vargiami E, Mayapetek E, Augoustidou-Savvopoulou P, Mitchell GA. Â In this paper, the authors describe a patient with HMG-CoA Lyase deficiency. He initially presented at 8 months with seizures during a gastroenteritis; he had hepatomegaly and elevated […]. ...
The background to it is that UK has like 3 major TV/Cable/Phone providers (like Starhub back home) - British Telecom, Sky and Virgin Media. The rest of the telcos like Vodafone, O2 etc do some mobile and internet but not that comprehensive. Since there are these major providers who do everything, youd think that you can just call up one company and get it all done in one installation and one bill right? WRONG. Apparently, whilst everyone has 3-in-1 packages, for most of the internet here, its all through ADSL. And what does ADSL need? An active phone line. And heres the punchline - only BT provides the phone line. So that means that before you can do anything at all with anyone, you have to get BT to activate your phone line (and pay them while at it, this is the hidden cost that NO ONE tells you when you see all those cheap packages - £11/mth!!), which is why everyone ends up getting BT internet and phone. I was totally resisting this monopoly cos I really dont think I need a home phone ...
Security updates or virus definitions? What first? I am connecting a Win2k PC, stand alone, to the Internet for the first time. ADSL. ...
Skys successful drive into broadband is set to shift up a gear in November when it will launch a package aimed at the UKs 10 million-plus Freeview households.. Sky Broadband launched last year after the group bought Easynet, but currently only supplies existing TV customers as part of a bundle. It offers up to theoretical 16MBit/s ADSL via the Easynet local loop unbundled network for £10 per month. Non-TV customers can expect to pay a bit more.. Multiple sources inside the company have indicated that Sky will pitch its standalone ISP service under a new brand called Picnic. Customers will be supplied with a Netgear DG834GT v4 router, which is currently being tested by Skys engineers for any glitches when connected to its network.. Internet "brand protection" outfit MarkMonitor has registered a series of domains including picnicbroadband.co.uk and picnicuser.co.uk. MarkMonitor ran a similar landgrab operation for Skys bundled broadband service.. We contacted Sky for their response to the ...
Your network card has an IP and your modem has an IP address if you connect to the Internet, but both is diffident because your LAN is not part of the internet. When you connect to Internet, depending on your connection, you get a fixed ( static ) IP or a dynamic IP. Modem users gets a IP address that changed every time they reconnect to internet, Very useful if you attack people, so if you disconnect, then you get a diffident IP. If you have a ISDN or maybe ADSL connection most of the time you will get a static IP, so whenever you connect to internet you get the same IP every time. Attack someone without IP spoofing, they will be able to see it is your IP attacking them! They can then go to your ISP ( internet service provider ), check who does this IP belong to and you can get into trouble. This is where modem users are lucky, they dont have a static IP and then can go mad and attack people and so without being caught, well almost... Their ISP can still check who was using the certain IP for ...
This step is catalyzed by adenylosuccinate lyase. Inosine monophosphate is converted to guanosine monophosphate by the ... First, GTP hydrolysis fuels the addition of aspartate to IMP by adenylosuccinate synthase, substituting the carbonyl oxygen for ... a nitrogen and forming the intermediate adenylosuccinate. Fumarate is then cleaved off forming adenosine monophosphate. ...
Adenylosuccinate lyase deficiency Extremophile Guthrie test Euzéby JP (2008). "Bacillus". List of Prokaryotic names with ...
... of a Mutant Bacillus subtilis Adenylosuccinate Lyase Equivalent to a Mutant Enzyme Found in Human Adenylosuccinate Lyase ... "Evaluation of Types of Interactions in Subunit Association in Bacillus subtilis Adenylosuccinate Lyase". Biochemistry. 47 (9): ...
GMP back into IMP adenylosuccinate synthase converts IMP to adenylosuccinate adenylosuccinate lyase converts adenylosuccinate ... CAIR + L-Aspartate + ATP → SAICAR + ADP + Pi The eight is catalyzed by adenylosuccinate lyase. SAICAR → AICAR + Fumarate The ...
... the Ethiopian male name Adenylosuccinate lyase. ...
... untranslated region of the ADSL gene in three patients with adenylosuccinate lyase deficiency". Am. J. Hum. Genet. 71 (1): 14- ...
... anemia due to Adenylosuccinate lyase deficiency Adie syndrome Adiposis dolorosa, aka Dercum's disease Adolescent benign focal ...
... a molecule whose appearance is characteristic of the disease adenylosuccinate lyase deficiency Saica may refer to : Saica ( ...
Adenylosuccinate + GDP + Pi Finally, Adenylosuccinate is cleaved by the enzyme adenylosuccinate lyase to release fumarate and ... to sustain mitochondrial membrane potential during anoxic stress by utilizing fumarate produced by adenylosuccinate lyase as an ... NH4+ The second stage is the formation of adenylosuccinate from IMP and the amino acid aspartate, which is coupled to the ... regenerate the starting material of AMP: Adenylosuccinate → AMP + Fumarate A recent study conducted by Sridharan et al. (AJP ...
... lyase deficiency Purine nucleotide cycle Figures 20.4 and 20.7 in Textbook of Biochemistry, with clinical ... Adenylosuccinate is an intermediate in the interconversion of purine nucleotides inosine monophosphate (IMP) and adenosine ... monophosphate (AMP). The enzyme adenylosuccinate synthase carries out the reaction by the addition of aspartate to IMP and ...
Adenylosuccinase, EC 4.3.2.2 (adenylosuccinate lyase), which catalyzes the eighth step in the de novo biosynthesis of purines, ... Aspartate ammonia-lyase, EC 4.3.1.1 (aspartase), which catalyzes the reversible conversion of aspartate to fumarate and ammonia ... A number of enzymes, belonging to the lyase class, for which fumarate is a substrate, have been shown to share a short ... Delta-crystallin shares around 90% sequence identity with arginosuccinate lyase, showing that it is an example of a 'hijacked' ...
... (or adenylosuccinase) is an enzyme that in humans is encoded by the ADSL gene. Adenylosuccinate lyase ... Mutated adenylosuccinate lyase (ASL) causes clinical disease in patients that is referred to as adenylosuccinate lyase ... "The characterization of mutant Bacillus subtilis adenylosuccinate lyases corresponding to severe human adenylosuccinate lyase ... of a mutant Bacillus subtilis adenylosuccinate lyase equivalent to a mutant enzyme found in human adenylosuccinate lyase ...
Adenylosuccinate Adenylosuccinate lyase List of genetic disorders "Adenylosuccinase deficiency". rarediseases.info.nih.gov. ... and adenylosuccinate (S-AMP), the two substrates of adenylosuccinate lyase (ADSL), which catalyzes an important reaction in the ... Treatment of adenylosuccinate lyase deficiency can be done via epilepsy management with anticonvulsive drugs.Additionally the ... "Adenylosuccinate lyase deficiency - Conditions - GTR - NCBI". www.ncbi.nlm.nih.gov. Retrieved 22 December 2016. RESERVED, ...
... tyrosine phenol-lyase MeSH D08.811.520.232.300 --- amidine-lyases MeSH D08.811.520.232.300.200 --- adenylosuccinate lyase MeSH ... oxo-acid-lyases MeSH D08.811.520.224.600.200 --- anthranilate synthase MeSH D08.811.520.224.600.700 --- isocitrate lyase MeSH ... ethanolamine ammonia-lyase MeSH D08.811.520.232.400.500 --- histidine ammonia-lyase MeSH D08.811.520.232.400.600 --- l-serine ... lyase MeSH D08.811.520.241.300 --- hydro-lyases MeSH D08.811.520.241.300.050 --- aconitate hydratase MeSH D08.811.520.241. ...
Adenylosuccinate synthase. *Adenylosuccinate lyase. *reverse *AMP deaminase. IMP→GMP:. *IMP dehydrogenase. *GMP synthase ...
Adenylosuccinate lyase deficiency. *Adenosine Monophosphate Deaminase Deficiency type 1. Nucleotide salvage. *Lesch-Nyhan ...
Adenylosuccinate synthase. *Adenylosuccinate lyase. *reverse *AMP deaminase. IMP→GMP:. *IMP dehydrogenase. *GMP synthase ...
Adenylosuccinate lyase deficiency. *Adenosine Monophosphate Deaminase Deficiency type 1. Nucleotide salvage. *Lesch-Nyhan ...
Adenylosuccinate lyase deficiency. *Adermatoglyphia. *Adhesive capsulitis of shoulder. *Adie syndrome. *Adipose tissue neoplasm ...
Adenylosuccinate lyase deficiency. *Pitt-Hopkins syndrome. *Phelan-McDermid syndrome. *Prader-Willi syndrome ...
... 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 ...
... 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. ... adenylosuccinate synthase EC 6.3.4.5: argininosuccinate synthase EC 6.3.4.6: urea carboxylase EC 6.3.4.7: ribose-5-phosphate- ...
Adenylosuccinate synthase. *Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. * ...
Adenylosuccinate synthase. *Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. * ...
Adenylosuccinate synthase. *Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. * ...
Adenylosuccinate synthase. *Argininosuccinate synthase. *Holocarboxylase synthetase. *GMP synthase. *Asparagine synthetase. * ...
Adenylosuccinate lyase (or adenylosuccinase) is an enzyme that in humans is encoded by the ADSL gene. Adenylosuccinate lyase ... Mutated adenylosuccinate lyase (ASL) causes clinical disease in patients that is referred to as adenylosuccinate lyase ... "The characterization of mutant Bacillus subtilis adenylosuccinate lyases corresponding to severe human adenylosuccinate lyase ... of a mutant Bacillus subtilis adenylosuccinate lyase equivalent to a mutant enzyme found in human adenylosuccinate lyase ...
... medlineplus.gov/genetics/condition/adenylosuccinate-lyase-deficiency/ Adenylosuccinate lyase deficiency. ... All forms of adenylosuccinate lyase deficiency are caused by mutations in the ADSL gene. This gene provides instructions for ... Adenylosuccinate lyase deficiency type I (also known as the severe form) is the most common. The signs and symptoms of this ... Adenylosuccinate lyase deficiency is a rare disorder; fewer than 100 cases have been reported. The condition is most common in ...
Adenylosuccinate Adenylosuccinate lyase List of genetic disorders "Adenylosuccinase deficiency". rarediseases.info.nih.gov. ... and adenylosuccinate (S-AMP), the two substrates of adenylosuccinate lyase (ADSL), which catalyzes an important reaction in the ... Treatment of adenylosuccinate lyase deficiency can be done via epilepsy management with anticonvulsive drugs.Additionally the ... "Adenylosuccinate lyase deficiency - Conditions - GTR - NCBI". www.ncbi.nlm.nih.gov. Retrieved 22 December 2016. RESERVED, ...
Definition of adenylosuccinate lyase. Provided by Stedmans medical dictionary and Drugs.com. Includes medical terms and ... adenylosuccinate lyase. Pronunciation: ad′e-nil-ō-sŭk′sin-āt lī′ās ...
Adenylosuccinate lyase C-terminal (IPR019468). Short name: AdenyloSucc_lyase_C Overlapping homologous superfamilies *L- ... Adenylosuccinate lyase catalyses two steps in the synthesis of purine nucleotides: the conversion of succinylaminoimidazole- ... This entry represents the seven alpha-helical, C-terminal domain of adenylosuccinate lyase [PMID: 9274883]. It is also found in ... Adenylosuccinate lyase deficiency: the first identified polish patient.. Brain Dev. 29 600-2 2007 ...
IPR022761 Fumarate_lyase_N. IPR008948 L-Aspartase-like. IPR004769 Pur_lyase. Pfami. View protein in Pfam. PF00206 Lyase_1, 1 ... IPR020557 Fumarate_lyase_CS. IPR000362 Fumarate_lyase_fam. IPR022761 Fumarate_lyase_N. IPR008948 L-Aspartase-like. IPR004769 ... Adenylosuccinate lyaseImported. ,p>Information which has been imported from another database using automatic procedures.,/p> ,p ... tr,E9Q3T7,E9Q3T7_MOUSE Adenylosuccinate lyase OS=Mus musculus OX=10090 GN=Adsl PE=1 SV=1 ...
Adenylosuccinate lyase (purB), Adenylosuccinate lyase (purB_1), Adenylosuccinate lyase (pcaB_2), Adenylosuccinate lyase (EA770_ ... Adenylosuccinate lyase (purB), Adenylosuccinate lyase (purB_1), Adenylosuccinate lyase (pcaB_2), Adenylosuccinate lyase (EA770_ ... 10675), Adenylosuccinate lyase (purB), Adenylosuccinate lyase (purB_1), Adenylosuccinate lyase (purB_2), Adenylosuccinate lyase ... 10675), Adenylosuccinate lyase (purB), Adenylosuccinate lyase (purB_1), Adenylosuccinate lyase (purB_2), Adenylosuccinate lyase ...
The crystal structure of adenylosuccinate lyase from Pyrobaculum aerophilum reveals an intracellular protein with three ... ADENYLOSUCCINATE LYASE A 431 Bacillus subtilis EC#: 4.3.2.2 IUBMB Gene Name(s): purB purE BSU06440 ...
The crystal structure of adenylosuccinate lyase from Pyrobaculum aerophilum reveals an intracellular protein with three ... Adenylosuccinate lyase 1392 4.3.2.2 , Details 4 5HW2 1 A, B, C, D Adenylosuccinate lyase 263 4.3.2.2 , Details ... Adenylosuccinate lyase 263 4.3.2.2 , Details 3 2PFM 1 A, B ... ADENYLOSUCCINATE LYASE 13773 4.3.2.2 , Details 8 5XNZ 1 A CreD ... PROTEIN (ADENYLOSUCCINATE LYASE) 2336 4.3.2.2 , Details 2 4EEI 1 A, B ...
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... catalysing the nonhydrolytic cleavage of succinyl groups from SAICA ribotide and adenylosuccinate. Enzyme defects are ... Adenylosuccinate lyase is an enzyme of fumarase superfamily that participates in the purine biosynthetic pathway, ... Inhibition of defective adenylosuccinate lyase by HNE: A neurological disease that may be affected by oxidative stress Issue ... Adenylosuccinate lyase activity is lost to a different extent in the patients. Diminished levels of enzyme have been attributed ...
Human Adenylosuccinate lyase (ADSL) ELISA Kit-YP_004341812.1 (MBS7236022) product datasheet at MyBioSource, ELISA Kits ... ADSL elisa kit :: Human Adenylosuccinate lyase (ADSL) ELISA Kit. Catalog #. MBS7236022 .mycenter { display: block; margin-left ... Kit for analyzing the presence of the Adenylosuccinate lyase (ADSL) ELISA Kit target analytes in biological samples. The ... Adenylosuccinate lyase (ADSL), ELISA Kit. ★Popular Item★ Also Known As Human Adenylosuccinate lyase (ADSL) ELISA Kit. ...
Adenylosuccinate lyase deficiency. More than 50 mutations in the ADSL gene have been found to cause adenylosuccinate lyase ... A reduction of adenylosuccinate lyase function, possibly due to a shortage of purinosomes, leads to buildup of SAICAR and SAMP ... Adenylosuccinate lyase and other enzymes involved in purine synthesis form a group of proteins (a protein complex) called the ... The ADSL gene provides instructions for making an enzyme called adenylosuccinate lyase. This enzyme performs two steps in the ...
Adenylosuccinate lyase,Adl,Adsl,ASase,ASL,Mouse,Mus musculus \ EIAAB33171 for more molecular products just contact us ... Adenylosuccinate lyase,Adl,Adsl,ASase,ASL,Mouse,Mus musculus mus musculus murine Adenylosuccinase,Adenylosuccinate lyase,Adl, ... Adenylosuccinate lyase,Adl,Adsl,ASase,ASL,Mouse,Mus musculus. Related products : Adenylosuccinase,Adenylosuccinate lyase,Adl, ... Adenylosuccinate lyase,Adl,Adsl,ASase,ASL,Mouse,Mus musculus / Product Detail : EIAAB33171 Adenylosuccinase,Adenylosuccinate ...
Showing Protein Adenylosuccinate lyase (BMDBP00935). IdentificationBiological propertiesGene propertiesProtein properties ...
Get natural cures for Adenylosuccinate lyase deficiency that can make a difference in your life or the life of someone you love ... Adenylosuccinate lyase deficiency by state. Adenylosuccinate lyase deficiency in Alabama. Adenylosuccinate lyase deficiency in ... Adenylosuccinate lyase deficiency in Iowa. Adenylosuccinate lyase deficiency in Kansas. Adenylosuccinate lyase deficiency in ... Adenylosuccinate lyase deficiency in North Dakota. Adenylosuccinate lyase deficiency in Ohio. Adenylosuccinate lyase deficiency ...
adenylosuccinate lyase. Enable Javascript to view the expand/collapse boxes.. Printable PDF Open All Close All ... OMIM: ADENYLOSUCCINATE LYASE. *. Ariyananda Lde Z, Lee P, Antonopoulos C, Colman RF. Biochemical and biophysical analysis of ... A reduction of adenylosuccinate lyase function, possibly due to a shortage of purinosomes, leads to buildup of SAICAR and SAMP ... Adenylosuccinate lyase and other enzymes involved in purine synthesis form a group of proteins (a protein complex) called the ...
Adenylosuccinate Lyase, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human ... orf clones - Search for available Adenylosuccinate Lyase ORF products * Overview of 142 available Adenylosuccinate Lyase gene ... orf clones - Search for available Adenylosuccinate Lyase ORF products * Overview of 142 available Adenylosuccinate Lyase gene ... orf clones - Search for available Adenylosuccinate Lyase ORF products. *Overview of 142 available Adenylosuccinate Lyase gene ...
... and Arg301 are critical for catalysis by adenylosuccinate lyase from Bacillus subtilis. ... and Arg301 are critical for catalysis by adenylosuccinate lyase from Bacillus subtilis. In adenylosuccinate lyase from Bacillus ... 15182182] Gln212, Asn270, and Arg301 are critical for catalysis by adenylosuccinate lyase from Bacillus subtilis.. ... metC yjcJ BSU11880] Cystathionine beta-lyase MetC (CBL) (EC 4.4.1.13) (Beta-cystathionase MetC) (Cysteine lyase MetC) (Cysteine ...
Adenylosuccinate Lyase Deficiency (disorder) (2). *. Atrophy (2). *. Autistic Disorder (2). *. Cholangiocarcinoma (2) ...
Adenylosuccinate lyase. Saccharomyces cerevisiae (strain ATCC 204508 / S288c) (Bakers yeast) ... Adenylosuccinate lyase UniProtKBInterProSTRINGInteractive Modelling. 482 aa; Sequence (Fasta) Identical sequences: ...
... adenylosuccinate lyase ...
Adenylosuccinate Lyase 4.3.2.2. ADP-Ribosylcyclase none. Agarase 3.2.1.81. Alanine Aminopeptidase 3.4.11.14. ...
adenylosuccinate lyase. MGI:103202 Go Annotations as Summary Text (Tabular View) (GO Graph). Automated description from the ... Orthologous to human ADSL (adenylosuccinate lyase).. Go Annotations in Tabular Form (Text View) (GO Graph) Filter annotations ... Predicted to have (S)-2-(5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido)succinate AMP-lyase (fumarate-forming) activity ... Human ortholog(s) of this gene implicated in adenylosuccinase lyase deficiency. ...
adenylosuccinate synthase converts IMP to adenylosuccinate. *adenylosuccinate lyase converts adenylosuccinate into AMP ...
  • The mutations include those in purB (adenylosuccinate lyase, adenine requirement) ( 16 ) and icdA (isocitrate dehydrogenase, glutamate requirement) ( 17 ) and others causing requirements for methionine or cysteine ( 17 ). (asm.org)
  • This entry represents the seven alpha-helical, C-terminal domain of adenylosuccinate lyase [ PMID: 9274883 ]. (ebi.ac.uk)
  • The structure, function and reaction mechanism of several PLP-dependent enzymes including serinehydroxymethyl transferase, actetylornithine aminotransferase, D-serine deaminase, D-cysteine desulfhydrase, arginine decarboxylase, pyridoxal kinase and diaminopropionate ammonia lyase have been investigated. (ibab.ac.in)
  • It was previously thought that the mechanism of action for adenylosuccinate lyase was a concerted catalysis where the hydrogen on the β-carbon (with respect to the leaving nitrogen) was abstracted by the catalytic base at the same time that the leaving nitrogen was protonated by the catalytic acid for E2 elimination. (wikipedia.org)
  • Adenylosuccinate lyase is part of the β-elimination superfamily of enzymes and it proceeds through an E1cb reaction mechanism. (wikipedia.org)
  • It is suggested that inactivation of defective adenylosuccinate lyase by HNE and other membrane peroxidation products may account, at least in part, for the impairment of neurological functions and recurrent worsening of the symptoms. (iospress.com)
  • Amino Acids of the Active Site of Adenylosuccinate Lyase. (udel.edu)
  • Colman's lab studies the enzyme glutathione S-transferase, which plays an important role in detoxifying foreign chemicals, and adenylosuccinate lyase, a shortage of which is related to mental retardation and autism. (asbmb.org)