Adenosine Deaminase: An enzyme that catalyzes the hydrolysis of ADENOSINE to INOSINE with the elimination of AMMONIA.Adenosine Deaminase Inhibitors: Drugs that inhibit ADENOSINE DEAMINASE activity.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.Nucleoside Deaminases: Catalyze the hydrolysis of nucleosides with the elimination of ammonia.Coformycin: A ribonucleoside antibiotic synergist and adenosine deaminase inhibitor isolated from Nocardia interforma and Streptomyces kaniharaensis. It is proposed as an antineoplastic synergist and immunosuppressant.AMP Deaminase: An enzyme that catalyzes the deamination of AMP to IMP. EC 3.5.4.6.Pentostatin: A potent inhibitor of ADENOSINE DEAMINASE. The drug induces APOPTOSIS of LYMPHOCYTES, and is used in the treatment of many lymphoproliferative malignancies, particularly HAIRY CELL LEUKEMIA. It is also synergistic with some other antineoplastic agents and has immunosuppressive activity.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.Adenosine Kinase: An enzyme that catalyzes the formation of ADP plus AMP from adenosine plus ATP. It can serve as a salvage mechanism for returning adenosine to nucleic acids. EC 2.7.1.20.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.Cytidine Deaminase: An enzyme that catalyzes the deamination of cytidine, forming uridine. EC 3.5.4.5.Deoxyadenosines: Adenosine molecules which can be substituted in any position, but are lacking one hydroxyl group in the ribose part of the molecule.Inosine: A purine nucleoside that has hypoxanthine linked by the N9 nitrogen to the C1 carbon of ribose. It is an intermediate in the degradation of purines and purine nucleosides to uric acid and in pathways of purine salvage. It also occurs in the anticodon of certain transfer RNA molecules. (Dorland, 28th ed)Receptors, Purinergic P1: A class of cell surface receptors that prefer ADENOSINE to other endogenous PURINES. Purinergic P1 receptors are widespread in the body including the cardiovascular, respiratory, immune, and nervous systems. There are at least two pharmacologically distinguishable types (A1 and A2, or Ri and Ra).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.Cytosine Deaminase: An enzyme which catalyzes the deamination of CYTOSINE resulting in the formation of URACIL. It can also act on 5-methylcytosine to form THYMIDINE.DCMP Deaminase: An enzyme that catalyzes the hydrolytic deamination of deoxycytidylic acid to deoxyuridylic acid and ammonia. It plays an important role in the regulation of the pool of deoxynucleotides in higher organisms. The enzyme also acts on some 5-substituted deoxycytidylic acids. EC 3.5.4.12.Receptor, Adenosine A2B: A subclass of adenosine A2 receptors found in the CECUM, the COLON, the BLADDER, and a variety of other tissues. It is generally considered to be a low affinity receptor for ADENOSINE that couples to the GS, STIMULATORY G-PROTEIN.Ribonucleosides: Nucleosides in which the purine or pyrimidine base is combined with ribose. (Dorland, 28th ed)Guanine Deaminase: An enzyme that catalyzes the deamination of guanine to form xanthine. EC 3.5.4.3.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.Xanthines: Purine bases found in body tissues and fluids and in some plants.Adenosine A2 Receptor Antagonists: Compounds that selectively bind to and block the activation of ADENOSINE A2 RECEPTORS.Tuberculosis, Pleural: Tuberculosis of the serous membrane lining the thoracic cavity and surrounding the lungs.Tubercidin: An antibiotic purine ribonucleoside that readily substitutes for adenosine in the biological system, but its incorporation into DNA and RNA has an inhibitory effect on the metabolism of these nucleic acids.5'-Nucleotidase: A glycoprotein enzyme present in various organs and in many cells. The enzyme catalyzes the hydrolysis of a 5'-ribonucleotide to a ribonucleoside and orthophosphate in the presence of water. It is cation-dependent and exists in a membrane-bound and soluble form. EC 3.1.3.5.Adenosine A2 Receptor Agonists: Compounds that selectively bind to and activate ADENOSINE A2 RECEPTORS.Nucleotide Deaminases: Catalyze the hydrolysis of nucleotides with the elimination of ammonia.Purinergic P1 Receptor Antagonists: Compounds that bind to and block the stimulation of PURINERGIC P1 RECEPTORS.Adenosine A1 Receptor Antagonists: Compounds that bind to and block the stimulation of ADENOSINE A1 RECEPTORS.RNA Editing: A process that changes the nucleotide sequence of mRNA from that of the DNA template encoding it. Some major classes of RNA editing are as follows: 1, the conversion of cytosine to uracil in mRNA; 2, the addition of variable number of guanines at pre-determined sites; and 3, the addition and deletion of uracils, templated by guide-RNAs (RNA, GUIDE).Adenosine A1 Receptor Agonists: Compounds that bind to and stimulate ADENOSINE A1 RECEPTORS.Purinergic P1 Receptor Agonists: Compounds that bind to and stimulate PURINERGIC P1 RECEPTORS.Phenylisopropyladenosine: N-Isopropyl-N-phenyl-adenosine. Antilipemic agent. Synonym: TH 162.Purine-Nucleoside Phosphorylase: An enzyme that catalyzes the reaction between a purine nucleoside and orthophosphate to form a free purine plus ribose-5-phosphate. EC 2.4.2.1.Adenosine Monophosphate: Adenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position.AminohydrolasesImmunologic Deficiency Syndromes: Syndromes in which there is a deficiency or defect in the mechanisms of immunity, either cellular or humoral.Adenine: A purine base and a fundamental unit of ADENINE NUCLEOTIDES.Deamination: The removal of an amino group (NH2) from a chemical compound.Theophylline: A methyl xanthine derivative from tea with diuretic, smooth muscle relaxant, bronchial dilation, cardiac and central nervous system stimulant activities. Theophylline inhibits the 3',5'-CYCLIC NUCLEOTIDE PHOSPHODIESTERASE that degrades CYCLIC AMP thus potentiates the actions of agents that act through ADENYLYL CYCLASES and cyclic AMP.2-Chloroadenosine: 2-Chloroadenosine. A metabolically stable analog of adenosine which acts as an adenosine receptor agonist. The compound has a potent effect on the peripheral and central nervous system.Pleural Effusion: Presence of fluid in the pleural cavity resulting from excessive transudation or exudation from the pleural surfaces. It is a sign of disease and not a diagnosis in itself.Dipeptidyl Peptidase 4: A serine protease that catalyses the release of an N-terminal dipeptide. Several biologically-active peptides have been identified as dipeptidyl peptidase 4 substrates including INCRETINS; NEUROPEPTIDES; and CHEMOKINES. The protein is also found bound to ADENOSINE DEAMINASE on the T-CELL surface and is believed to play a role in T-cell activation.Adenosine-5'-(N-ethylcarboxamide): A stable adenosine A1 and A2 receptor agonist. Experimentally, it inhibits cAMP and cGMP phosphodiesterase activity.Deoxyadenine Nucleotides: Adenine nucleotides which contain deoxyribose as the sugar moiety.Severe Combined Immunodeficiency: Group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody levels. It is inherited as an X-linked or autosomal recessive defect. Mutations occurring in many different genes cause human Severe Combined Immunodeficiency (SCID).Purine-Pyrimidine Metabolism, Inborn ErrorsReceptors, Purinergic: Cell surface proteins that bind PURINES with high affinity and trigger intracellular changes which influence the behavior of cells. The best characterized classes of purinergic receptors in mammals are the P1 receptors, which prefer ADENOSINE, and the P2 receptors, which prefer ATP or ADP.Nucleotidases: A class of enzymes that catalyze the conversion of a nucleotide and water to a nucleoside and orthophosphate. EC 3.1.3.-.Electrophoresis, Starch Gel: Electrophoresis in which a starch gel (a mixture of amylose and amylopectin) is used as the diffusion medium.Adenine NucleotidesHydroxymethylbilane Synthase: An enzyme that catalyzes the tetrapolymerization of the monopyrrole PORPHOBILINOGEN into the hydroxymethylbilane preuroporphyrinogen (UROPORPHYRINOGENS) in several discrete steps. It is the third enzyme in the 8-enzyme biosynthetic pathway of HEME. In humans, deficiency in this enzyme encoded by HMBS (or PBGD) gene results in a form of neurological porphyria (PORPHYRIA, ACUTE INTERMITTENT). This enzyme was formerly listed as EC 4.3.1.8Adenosylhomocysteinase: An enzyme which catalyzes the catabolism of S-ADENOSYLHOMOCYSTEINE to ADENOSINE and HOMOCYSTEINE. It may play a role in regulating the concentration of intracellular adenosylhomocysteine.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.Hypoxanthines: Purine bases related to hypoxanthine, an intermediate product of uric acid synthesis and a breakdown product of adenine catabolism.Hypoxanthine: A purine and a reaction intermediate in the metabolism of adenosine and in the formation of nucleic acids by the salvage pathway.Pentosyltransferases: Enzymes of the transferase class that catalyze the transfer of a pentose group from one compound to another.Phenethylamines: A group of compounds that are derivatives of beta- aminoethylbenzene which is structurally and pharmacologically related to amphetamine. (From Merck Index, 11th ed)Kinetics: The rate dynamics in chemical or physical systems.Clinical Enzyme Tests: Analyses for a specific enzyme activity, or of the level of a specific enzyme that is used to assess health and disease risk, for early detection of disease or disease prediction, diagnosis, and change in disease status.Nucleosides: Purine or pyrimidine bases attached to a ribose or deoxyribose. (From King & Stansfield, A Dictionary of Genetics, 4th ed)Cyclic AMP: An adenine nucleotide containing one phosphate group which is esterified to both the 3'- and 5'-positions of the sugar moiety. It is a second messenger and a key intracellular regulator, functioning as a mediator of activity for a number of hormones, including epinephrine, glucagon, and ACTH.Carbon-Carbon Lyases: Enzymes that catalyze the cleavage of a carbon-carbon bond by means other than hydrolysis or oxidation. This subclass contains the DECARBOXYLASES, the ALDEHYDE-LYASES, and the OXO-ACID-LYASES. EC 4.1.Purine Nucleosides: Purines with a RIBOSE attached that can be phosphorylated to PURINE NUCLEOTIDES.Leukemia, Lymphoid: Leukemia associated with HYPERPLASIA of the lymphoid tissues and increased numbers of circulating malignant LYMPHOCYTES and lymphoblasts.Erythrocytes: Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN.Adenosine A3 Receptor Antagonists: Compounds that selectively bind to and block the activation of ADENOSINE A3 RECEPTORS.Dipyridamole: A phosphodiesterase inhibitor that blocks uptake and metabolism of adenosine by erythrocytes and vascular endothelial cells. Dipyridamole also potentiates the antiaggregating action of prostacyclin. (From AMA Drug Evaluations Annual, 1994, p752)Pericarditis, Tuberculous: INFLAMMATION of the sac surrounding the heart (PERICARDIUM) due to MYCOBACTERIUM TUBERCULOSIS infection. Pericarditis can lead to swelling (PERICARDIAL EFFUSION), compression of the heart (CARDIAC TAMPONADE), and preventing normal beating of the heart.Base Sequence: The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.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.Thioinosine: Sulfhydryl analog of INOSINE that inhibits nucleoside transport across erythrocyte plasma membranes, and has immunosuppressive properties. It has been used similarly to MERCAPTOPURINE in the treatment of leukemia. (From Martindale, The Extra Pharmacopoeia, 30th ed, p503)Threonine Dehydratase: A pyridoxal-phosphate protein that catalyzes the deamination of THREONINE to 2-ketobutyrate and AMMONIA. The role of this enzyme can be biosynthetic or biodegradative. In the former role it supplies 2-ketobutyrate required for ISOLEUCINE biosynthesis, while in the latter it is only involved in the breakdown of threonine to supply energy. This enzyme was formerly listed as EC 4.2.1.16.Cell Line: Established cell cultures that have the potential to propagate indefinitely.Adenosine A3 Receptor Agonists: Drugs that selectively bind to and activate ADENOSINE A3 RECEPTORS.Cells, Cultured: Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.Peritonitis, Tuberculous: A form of PERITONITIS seen in patients with TUBERCULOSIS, characterized by lesion either as a miliary form or as a pelvic mass on the peritoneal surfaces. Most patients have ASCITES, abdominal swelling, ABDOMINAL PAIN, and other systemic symptoms such as FEVER; WEIGHT LOSS; and ANEMIA.Flucytosine: A fluorinated cytosine analog that is used as an antifungal agent.Deoxyribonucleosides: A purine or pyrimidine base bonded to DEOXYRIBOSE.Dideoxyadenosine: A dideoxynucleoside compound in which the 3'-hydroxy group on the sugar moiety has been replaced by a hydrogen. This modification prevents the formation of phosphodiester linkages which are needed for the completion of nucleic acid chains. The compound is an inhibitor of HIV replication, acting as a chain-terminator of viral DNA by binding to reverse transcriptase. Its principal side effect is nephrotoxicity. In vivo, dideoxyadenosine is rapidly metabolized to DIDANOSINE (ddI) by enzymatic deamination; ddI is then converted to dideoxyinosine monophosphate and ultimately to dideoxyadenosine triphosphate, the putative active metabolite.Formycins: Pyrazolopyrimidine ribonucleosides isolated from Nocardia interforma. They are antineoplastic antibiotics with cytostatic properties.Vidarabine: A nucleoside antibiotic isolated from Streptomyces antibioticus. It has some antineoplastic properties and has broad spectrum activity against DNA viruses in cell cultures and significant antiviral activity against infections caused by a variety of viruses such as the herpes viruses, the VACCINIA VIRUS and varicella zoster virus.S-Adenosylhomocysteine: 5'-S-(3-Amino-3-carboxypropyl)-5'-thioadenosine. Formed from S-adenosylmethionine after transmethylation reactions.Lymphocytes: White blood cells formed in the body's lymphoid tissue. The nucleus is round or ovoid with coarse, irregularly clumped chromatin while the cytoplasm is typically pale blue with azurophilic (if any) granules. Most lymphocytes can be classified as either T or B (with subpopulations of each), or NATURAL KILLER CELLS.Inosine Monophosphate: Inosine 5'-Monophosphate. A purine nucleotide which has hypoxanthine as the base and one phosphate group esterified to the sugar moiety.Purinergic Antagonists: Drugs that bind to and block the activation of PURINERGIC RECEPTORS.Tetrahydrouridine: An inhibitor of nucleotide metabolism.RNA, Double-Stranded: RNA consisting of two strands as opposed to the more prevalent single-stranded RNA. Most of the double-stranded segments are formed from transcription of DNA by intramolecular base-pairing of inverted complementary sequences separated by a single-stranded loop. Some double-stranded segments of RNA are normal in all organisms.DNA: A deoxyribonucleotide polymer that is the primary genetic material of all cells. Eukaryotic and prokaryotic organisms normally contain DNA in a double-stranded state, yet several important biological processes transiently involve single-stranded regions. DNA, which consists of a polysugar-phosphate backbone possessing projections of purines (adenine and guanine) and pyrimidines (thymine and cytosine), forms a double helix that is held together by hydrogen bonds between these purines and pyrimidines (adenine to thymine and guanine to cytosine).Pigmentation DisordersSomatic Hypermutation, Immunoglobulin: A programmed mutation process whereby changes are introduced to the nucleotide sequence of immunoglobulin gene DNA during development.Mutation: Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.Genes: A category of nucleic acid sequences that function as units of heredity and which code for the basic instructions for the development, reproduction, and maintenance of organisms.Guanosine: A purine nucleoside that has guanine linked by its N9 nitrogen to the C1 carbon of ribose. It is a component of ribonucleic acid and its nucleotides play important roles in metabolism. (From Dorland, 28th ed)Cloning, Molecular: 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.Lipid Mobilization: LIPOLYSIS of stored LIPIDS in the ADIPOSE TISSUE to release FREE FATTY ACIDS. Mobilization of stored lipids is under the regulation of lipolytic signals (CATECHOLAMINES) or anti-lipolytic signals (INSULIN) via their actions on the hormone-sensitive LIPASE. This concept does not include lipid transport.RNA, Messenger: RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.Triazines: Heterocyclic rings containing three nitrogen atoms, commonly in 1,2,4 or 1,3,5 or 2,4,6 formats. Some are used as HERBICIDES.Immunoglobulin Class Switching: Gene rearrangement of the B-lymphocyte which results in a substitution in the type of heavy-chain constant region that is expressed. This allows the effector response to change while the antigen binding specificity (variable region) remains the same. The majority of class switching occurs by a DNA recombination event but it also can take place at the level of RNA processing.Ammonia-Lyases: Enzymes that catalyze the formation of a carbon-carbon double bond by the elimination of AMMONIA. EC 4.3.1.Adenosine Triphosphatases: A group of enzymes which catalyze the hydrolysis of ATP. The hydrolysis reaction is usually coupled with another function such as transporting Ca(2+) across a membrane. These enzymes may be dependent on Ca(2+), Mg(2+), anions, H+, or DNA.Deoxycytidine Kinase: An enzyme that catalyzes reversibly the phosphorylation of deoxycytidine with the formation of a nucleoside diphosphate and deoxycytidine monophosphate. Cytosine arabinoside can also act as an acceptor. All natural nucleoside triphosphates, except deoxycytidine triphosphate, can act as donors. The enzyme is induced by some viruses, particularly the herpes simplex virus (HERPESVIRUS HOMINIS). EC 2.7.1.74.Apyrase: A calcium-activated enzyme that catalyzes the hydrolysis of ATP to yield AMP and orthophosphate. It can also act on ADP and other nucleoside triphosphates and diphosphates. EC 3.6.1.5.L-Serine Dehydratase: A PYRIDOXAL-phosphate containing enzyme that catalyzes the dehydration and deamination of L-serine to form pyruvate. This enzyme was formerly listed as EC 4.2.1.13.DNA Nucleotidylexotransferase: A non-template-directed DNA polymerase normally found in vertebrate thymus and bone marrow. It catalyzes the elongation of oligo- or polydeoxynucleotide chains and is widely used as a tool in the differential diagnosis of acute leukemias in man. EC 2.7.7.31.Nucleoside Transport Proteins: Proteins involved in the transport of NUCLEOSIDES across cellular membranes.Lipolysis: The metabolic process of breaking down LIPIDS to release FREE FATTY ACIDS, the major oxidative fuel for the body. Lipolysis may involve dietary lipids in the DIGESTIVE TRACT, circulating lipids in the BLOOD, and stored lipids in the ADIPOSE TISSUE or the LIVER. A number of enzymes are involved in such lipid hydrolysis, such as LIPASE and LIPOPROTEIN LIPASE from various tissues.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.Phosphotransferases: A rather large group of enzymes comprising not only those transferring phosphate but also diphosphate, nucleotidyl residues, and others. These have also been subdivided according to the acceptor group. (From Enzyme Nomenclature, 1992) EC 2.7.Hydrolases: Any member of the class of enzymes that catalyze the cleavage of the substrate and the addition of water to the resulting molecules, e.g., ESTERASES, glycosidases (GLYCOSIDE HYDROLASES), lipases, NUCLEOTIDASES, peptidases (PEPTIDE HYDROLASES), and phosphatases (PHOSPHORIC MONOESTER HYDROLASES). EC 3.Retroviridae: Family of RNA viruses that infects birds and mammals and encodes the enzyme reverse transcriptase. The family contains seven genera: DELTARETROVIRUS; LENTIVIRUS; RETROVIRUSES TYPE B, MAMMALIAN; ALPHARETROVIRUS; GAMMARETROVIRUS; RETROVIRUSES TYPE D; and SPUMAVIRUS. A key feature of retrovirus biology is the synthesis of a DNA copy of the genome which is integrated into cellular DNA. After integration it is sometimes not expressed but maintained in a latent state (PROVIRUSES).

The RNA-editing enzyme ADAR1 is localized to the nascent ribonucleoprotein matrix on Xenopus lampbrush chromosomes but specifically associates with an atypical loop. (1/1316)

Double-stranded RNA adenosine deaminase (ADAR1, dsRAD, DRADA) converts adenosines to inosines in double-stranded RNAs. Few candidate substrates for ADAR1 editing are known at this point and it is not known how substrate recognition is achieved. In some cases editing sites are defined by basepaired regions formed between intronic and exonic sequences, suggesting that the enzyme might function cotranscriptionally. We have isolated two variants of Xenopus laevis ADAR1 for which no editing substrates are currently known. We demonstrate that both variants of the enzyme are associated with transcriptionally active chromosome loops suggesting that the enzyme acts cotranscriptionally. The widespread distribution of the protein along the entire chromosome indicates that ADAR1 associates with the RNP matrix in a substrate-independent manner. Inhibition of splicing, another cotranscriptional process, does not affect the chromosomal localization of ADAR1. Furthermore, we can show that the enzyme is dramatically enriched on a special RNA-containing loop that seems transcriptionally silent. Detailed analysis of this loop suggests that it might represent a site of ADAR1 storage or a site where active RNA editing is taking place. Finally, mutational analysis of ADAR1 demonstrates that a putative Z-DNA binding domain present in ADAR1 is not required for chromosomal targeting of the protein.  (+info)

The extracellular versus intracellular mechanisms of inhibition of TCR-triggered activation in thymocytes by adenosine under conditions of inhibited adenosine deaminase. (2/1316)

The absence or low levels of adenosine deaminase (ADA) in humans result in severe combined immunodeficiency (SCID), which is characterized by hypoplastic thymus, T lymphocyte depletion and autoimmunity. Deficiency of ADA causes increased levels of both intracellular and extracellular adenosine, although only the intracellular lymphotoxicity of accumulated adenosine is considered in the pathogenesis of ADA SCID. It is shown that extracellular but not intracellular adenosine selectively inhibits TCR-triggered up-regulation of activation markers and apoptotic events in thymocytes under conditions of ADA deficiency. The effects of intracellular adenosine are dissociated from effects of extracellular adenosine in experiments using an adenosine transporter blocker. We found that prevention of toxicity of intracellular adenosine led to survival of TCR-cross-linked thymocytes in long-term (4 days) assays, but it was not sufficient for normal T cell differentiation under conditions of inhibited ADA. Surviving TCR-cross-linked thymocytes had a non-activated phenotype due to extracellular adenosine-mediated, TCR-antagonizing signaling. Taken together the data suggest that both intracellular toxicity and signaling by extracellular adenosine may contribute to pathogenesis of ADA SCID. Accordingly, extracellular adenosine may act on thymocytes, which survived intracellular toxicity of adenosine during ADA deficiency by counteracting TCR signaling. This, in turn, could lead to failure of positive and negative selection of thymocytes, and to additional elimination of thymocytes or autoimmunity of surviving T cells.  (+info)

Nucleotide pool imbalance and adenosine deaminase deficiency induce alterations of N-region insertions during V(D)J recombination. (3/1316)

Template-independent nucleotide additions (N regions) generated at sites of V(D)J recombination by terminal deoxynucleotidyl transferase (TdT) increase the diversity of antigen receptors. Two inborn errors of purine metabolism, deficiencies of adenosine deaminase (ADA) and purine nucleoside phosphorylase (PNP), result in defective lymphoid development and aberrant pools of 2'-deoxynucleotides that are substrates for TdT in lymphoid precursors. We have asked whether selective increases in dATP or dGTP pools result in altered N regions in an extrachromosomal substrate transfected into T-cell or pre-B-cell lines. Exposure of the transfected cells to 2'-deoxyadenosine and an ADA inhibitor increased the dATP pool and resulted in a marked increase in A-T insertions at recombination junctions, with an overall decreased frequency of V(D)J recombination. Sequence analysis of VH-DH-JH junctions from the IgM locus in B-cell lines from ADA-deficient patients demonstrated an increase in A-T insertions equivalent to that found in the transfected cells. In contrast, elevation of dGTP pools, as would occur in PNP deficiency, did not alter the already rich G-C content of N regions. We conclude that the frequency of V(D)J recombination and the composition of N-insertions are influenced by increases in dATP levels, potentially leading to alterations in antigen receptors and aberrant lymphoid development. Alterations in N-region insertions may contribute to the B-cell dysfunction associated with ADA deficiency.  (+info)

A study of the genetical structure of the Cuban population: red cell and serum biochemical markers. (4/1316)

Gene frequencies of several red cell and serum gentic markers were determined in the three main racial groups--whites, mulattoes and Negroes--of the Cuban population. The results were used to estimate the relative contribution of Caucasian and Negro genes to the genetic makeup of these three groups and to calculate the frequencies of these genes in the general Cuban population.  (+info)

Adenosine deaminase activity in thymus and other human tissues. (5/1316)

Adenosine deaminase activity (ADA) has been estimated in human tissues. Levels in the thymus during childhood were very much higher than in any of the other 6 tissues studied. Intermediate activities were obtained from spleen and lymph nodes and also skin. Cerebral cortex, liver and kidney had relatively low levels. ADA activity in lymphocytes from peripheral blood was significantly increased after antigenic stimulation by TAB immunization. The available evidence appears to be consistent with T-lymphocyte growth and development in the thymus being dependant on ADA.  (+info)

Regulation of forestomach-specific expression of the murine adenosine deaminase gene. (6/1316)

The maturation of stratified squamous epithelium of the upper gastrointestinal tract is a highly ordered process of development and differentiation. Information on the molecular basis of this process is, however, limited. Here we report the identification of the first murine forestomach regulatory element using the murine adenosine deaminase (Ada) gene as a model. In the adult mouse, Ada is highly expressed in the terminally differentiated epithelial layer of upper gastrointestinal tract tissues. The data reported here represent the identification and detailed analysis of a 1. 1-kilobase (kb) sequence located 3.4-kb upstream of the transcription initiation site of the murine Ada gene, which is sufficient to target cat reporter gene expression to the forestomach in transgenic mice. This 1.1-kb fragment is capable of directing cat reporter gene expression mainly to the forestomach of transgenic mice, with a level comparable to the endogenous Ada gene. This expression is localized to the appropriate cell types, confers copy number dependence, and shows the same developmental regulation. Mutational analysis revealed the functional importance of multiple transcription factor-binding sites.  (+info)

Human RNA-specific adenosine deaminase ADAR1 transcripts possess alternative exon 1 structures that initiate from different promoters, one constitutively active and the other interferon inducible. (7/1316)

RNA-specific adenosine deaminase (ADAR1) catalyzes the deamination of adenosine to inosine in viral and cellular RNAs. Two size forms of the ADAR1 editing enzyme are known, an IFN-inducible approximately 150-kDa protein and a constitutively expressed N-terminally truncated approximately 110-kDa protein. We have now identified alternative exon 1 structures of human ADAR1 transcripts that initiate from unique promoters, one constitutively expressed and the other IFN inducible. Cloning and sequence analyses of 5'-rapid amplification of cDNA ends (RACE) cDNAs from human placenta established a linkage between exon 2 of ADAR1 and two alternative exon 1 structures, designated herein as exon 1A and exon 1B. Analysis of RNA isolated from untreated and IFN-treated human amnion cells demonstrated that exon 1B-exon 2 transcripts were synthesized in the absence of IFN and were not significantly altered in amount by IFN treatment. By contrast, exon 1A-exon 2 transcripts were IFN inducible. Transient transfection analysis with reporter constructs led to the identification of two functional promoters, designated PC and PI. Exon 1B transcripts were initiated from the PC promoter whose activity in transient transfection reporter assays was not increased by IFN treatment. The 107-nt exon 1B mapped 14.5 kb upstream of exon 2. The 201-nt exon 1A that mapped 5.4 kb upstream of exon 2 was initiated from the interferon-inducible PI promoter. These results suggest that two promoters, one IFN inducible and the other not, initiate transcription of the ADAR1 gene, and that alternative splicing of unique exon 1 structures to a common exon 2 junction generates RNA transcripts with the deduced coding capacity for either the constitutively expressed approximately 110-kDa ADAR1 protein (exon 1B) or the interferon-induced approximately 150-kDa ADAR1 protein (exon 1A).  (+info)

Long RNA hairpins that contain inosine are present in Caenorhabditis elegans poly(A)+ RNA. (8/1316)

Adenosine deaminases that act on RNA (ADARs) are RNA-editing enzymes that convert adenosine to inosine within double-stranded RNA. In the 12 years since the discovery of ADARs only a few natural substrates have been identified. These substrates were found by chance, when genomically encoded adenosines were identified as guanosines in cDNAs. To advance our understanding of the biological roles of ADARs, we developed a method for systematically identifying ADAR substrates. In our first application of the method, we identified five additional substrates in Caenorhabditis elegans. Four of those substrates are mRNAs edited in untranslated regions, and one is a noncoding RNA edited throughout its length. The edited regions are predicted to form long hairpin structures, and one of the RNAs encodes POP-1, a protein involved in cell fate decisions.  (+info)

Double-stranded RNA-specific adenosine deaminase is an enzyme that in humans is encoded by the ADAR gene (which stands for adenosine deaminase acting on RNA). Adenosine deaminases acting on RNA (ADAR) are enzymes responsible for binding to double stranded RNA (dsRNA) and converting adenosine (A) to inosine (I) by deamination. ADAR protein is a RNA-binding protein, which functions in RNA-editing through post-transcriptional modification of mRNA transcripts by changing the nucleotide content of the RNA. The conversion from A to I in the RNA disrupt the normal A:U pairing which makes the RNA unstable. Inosine is structurally similar to that of guanine (G) which leads to I to cytosine (C) binding. In RNA I functions the same as G in both translation and replication. Codon changes can arise from editing which may lead to changes in the coding sequences for proteins and their functions. Most editing site are found in noncoding regions of RNA such as untranslated regions (UTRs), Alu elements and long ...
International Journal of Clinical Biochemistry and Research-IJCBR-Print ISSN No:-2394-6369 Online ISSN No:-2394-6377Article DOI No:-10.18231/2394-6377.2018.0017,Estimation of serum Adenosine Deaminase (ADA) level in sickle cell disease (SCD) and its association with reticulocyte count in a rural population of Chhattisg
Double-stranded RNA adenosine deaminase (ADAR1) is an ubiquitous enzyme in metazoa that edits pre-mRNA changing adenosine to inosine in regions of double-stranded RNA. Zalpha, an N-terminal domain of human ADAR1 encompassing 76 amino acid residues, shows apparent specificity for the left-handed Z-DN …
Adenosine deaminase deficiency (also called ADA deficiency or ADA-SCID) is an autosomal recessive metabolic disorder that causes immunodeficiency. It occurs in fewer than one in 100,000 live births worldwide. It accounts for about 15% of all cases of severe combined immunodeficiency (SCID). ADA deficiency may be present in infancy, childhood, adolescence, or adulthood. Age of onset and severity is related to some 29 known genotypes associated with the disorder. The main symptoms of ADA deficiency are pneumonia, chronic diarrhea, and widespread skin rashes. Affected children also grow much more slowly than healthy children and some have developmental delay. Most individuals with ADA deficiency are diagnosed with SCID in the first 6 months of life. The enzyme adenosine deaminase is encoded by a gene on chromosome 20. ADA deficiency is inherited in an autosomal recessive manner. This means the defective gene responsible for the disorder is located on an autosome (chromosome 20 is an autosome), and ...
Diagnostic efficacy of adenosine deaminase levels in cerebrospinal fluid in patients of Tubercular meningitis: A comparison with PCR for Mycobacterium tuberculosis
Introduction: The use of biological markers in the diagnosis of tuberculous pleural effusion (TPE) is a breakthrough. Demonstration of elevated levels of Pleural fluid Adenosine deaminase (ADA), interferon-gamma (IFN-γ), tuberculous proteins/antibodies lysozme etc. have been proposed. Adenosine deaminase (ADA) estimation in pleural fluid has been shown as reliable biomarker specially when there is suspicion of tuberculosis. Detection of mycobacterium DNA by PCR is also a proposed test3,4. However India being a developing country with much of its people below poverty line cannot afford expensive tests like ELISA, PCR, IFN-γ. Hence, there is need for relatively cheaper and simple tests with feasibility and sensitivity going hand-in-hand5 TPE being proposed to be a delayed hypersensitive reaction and lymphocytes play a major role in the pathogenesis. With ,50% lymphocytes in the pleural fluid, combined criterion of lymphocyte to neutrophil ratio of ,0.75 with a raised ADA level increased the ...
RNA editing by deamination of adenosine to inosine is an evolutionarily conserved process involved in many cellular pathways, from alternative splicing to miRNA targeting. In humans, it is carried out by no less than three major adenosine deaminases acting on RNA (ADARs): ADAR1-p150, ADAR1-p110, and ADAR2. However, the first two derive from alternative splicing, so that it is currently impossible to delete ADAR1-p110 without also knocking out ADAR1-p150 expression. Furthermore, the expression levels of ADARs varies wildly among cell types, and no study has systematically explored the effect of each of these isoforms on the cell transcriptome. In this study, RNA immunoprecipitation (RIP)-sequencing on overexpressed ADAR isoforms tagged with green fluorescent protein (GFP) shows that each ADAR is associated with a specific set of differentially expressed genes, and that they each bind to distinct set of RNA targets. Our results show a good overlap with known edited transcripts, establishing RIP-seq as a
TY - JOUR. T1 - Structural basis for the growth factor activity of human adenosine deaminase ADA2. AU - Zavialov, Anton V.. AU - Yu, Xiaodi. AU - Spillmann, Dorothe. AU - Lauvau, Grégoire. AU - Zavialo, Andrey V.. PY - 2010/4/16. Y1 - 2010/4/16. N2 - Two distinct adenosine deaminases, ADA1 and ADA2, are found in humans. ADA1 has an important role in lymphocyte function and inherited mutations in ADA1 result in severe combined immunodeficiency. The recently isolated ADA2 belongs to the novel family of adenosine deaminase growth factors (ADGFs), which play an important role in tissue development. The crystal structures of ADA2 and ADA2 bound to a transition state analogue presented here reveal the structural basis of the catalytic/signaling activity of ADGF/ADA2 proteins. In addition to the catalytic domain, the structures discovered two ADGF/ADA2-specific domains of novel folds that mediate the protein dimerization and binding to the cell surface receptors. This complex architecture is in sharp ...
TY - JOUR. T1 - ADAR2 regulates RNA stability by modifying access of decay-promoting RNA-binding proteins. AU - Anantharaman, Aparna. AU - Tripathi, Vidisha. AU - Khan, Abid. AU - Yoon, Je Hyun. AU - Singh, Deepak K.. AU - Gholamalamdari, Omid. AU - Guang, Shuomeng. AU - Ohlson, Johan. AU - Wahlstedt, Helene. AU - Öhman, Marie. AU - Jantsch, Michael F.. AU - Conrad, Nicholas K.. AU - Ma, Jian. AU - Gorospe, Myriam. AU - Prasanth, Supriya G.. AU - Prasanth, Kannanganattu V.. PY - 2017/4/20. Y1 - 2017/4/20. N2 - Adenosine deaminases acting on RNA (ADARs) catalyze the editing of adenosine residues to inosine (A-to-I) within RNA sequences, mostly in the introns and UTRs (un-translated regions). The significance of editing within non-coding regions of RNA is poorly understood. Here, we demonstrate that association of ADAR2 with RNA stabilizes a subset of transcripts. ADAR2 interacts with and edits the 3Î.,UTR of nuclear-retained Cat2 transcribed nuclear RNA (Ctn RNA). In absence of ADAR2, the ...
Preeclampsia is a prevalent pregnancy hypertensive disease with both maternal and fetal morbidity and mortality. Emerging evidence indicates that global placental DNA hypomethylation is observed in patients with preeclampsia and is linked to altered gene expression and disease development. However, the molecular basis underlying placental epigenetic changes in preeclampsia remains unclear. Using 2 independent experimental models of preeclampsia, adenosine deaminase-deficient mice and a pathogenic autoantibody-induced mouse model of preeclampsia, we demonstrate that elevated placental adenosine not only induces hallmark features of preeclampsia but also causes placental DNA hypomethylation. The use of genetic approaches to express an adenosine deaminase minigene specifically in placentas, or adenosine deaminase enzyme replacement therapy, restored placental adenosine to normal levels, attenuated preeclampsia features, and abolished placental DNA hypomethylation in adenosine deaminase-deficient ...
Abstract: RNA editing by the adenosine deaminase acting on RNA (ADAR) enzymes has been associated with many human neurological diseases including: epilepsy; suicidal depression; autism; pediatric glioblastoma; and ALS (Lou Gehrigs disease). RNA editing is ubiquitous in the animal kingdom. ADAR deaminates the RNA base adenosine (A) to inosine (I) in dsRNA molecules. Inosine is recognized by all cellular machineries as guanosine (G). ADAR specifically edits, recodes, a small number of adenosines in messenger RNA (mRNA) to such "Gs". However, hyper editing acts more generally on perfect or nearly perfect double-stranded RNA (dsRNA). Within long dsRNA (,30bp), over 40% of adenosine residues are modified on both strands, generating numerous I-U mismatch pairs, and structurally destabilizing dsRNA. Dicer is an enzyme that cleaves near perfect long dsRNAs, and thus competes with ADAR. As a consequence, ADARs hyper editing has downstream consequences on Dicer products including gene expression ...
One particular kind of SCID, called adenosine deaminase deficiency (ADA)-SCID, is caused by lack of an enzyme (a protein in the body that helps break down other chemicals). Patients with ADA-SCID typically have very low T-cells, B-cells, and NK-cells because toxic byproducts build up as result of lack of the ADA enzyme. Patients with ADA-SCID present with similar infections as seen with the other forms of SCID.. ADA-SCID is the only type of SCID where patients can receive enzyme replacement. The enzyme has been made into a drug known as PEG-ADA (Adagen ®). At the present time, PEG-ADA comes from cows (bovine), although attempts are underway to make a recombinant form that does not come from animals. PEG-ADA is given by a needle into the muscle (intramuscularly). Patients / parents learn to inject it themselves. Usually it is given once per week, although dose changes (both in terms of total dose and the frequency with which PEG-ADA is administered) may need to occur based upon ADA levels that ...
Synonyms for adenosine deaminase conjugated with polyethylene glycol in Free Thesaurus. Antonyms for adenosine deaminase conjugated with polyethylene glycol. 2 words related to adenosine: biochemistry, nucleoside. What are synonyms for adenosine deaminase conjugated with polyethylene glycol?
Both TAR DNA binding protein of 43kDa (TDP-43) pathology and failure of RNA editing at the glutamine/arginine (Q/R) site of GluA2, a subunit of the α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) receptor, are the characteristic etiology-linked molecular abnormalities that concomitantly occur in the motor neurons of the majority of patients with amyotrophic lateral sclerosis (ALS), the most common adult-onset fatal motor neuron disease. Adenosine deaminase acting on RNA 2 (ADAR2) specifically catalyzes RNA editing at the Q/R site of GluA2, and conditional ADAR2 knockout mice (ADAR2flox/flox/VAChT-Cre.Fast ; AR2 mice) exhibit a progressive ALS phenotype with TDP-43 pathology-like TDP-43 mislocalization in the ADAR2-lacking motor neurons. Because Ca2+-permeable AMPA receptor-mediated mechanism underlies death of motor neurons in the AR2 mice, amelioration of exaggerated Ca2+ influx by AMPA receptor antagonists may be a potential ALS therapy. Here we showed that oral perampanel, a selective
A collection of disease information resources and questions answered by our Genetic and Rare Diseases Information Specialists for Adenosine deaminase deficiency
Background: Adenosine deaminase acting on RNA-2 (ADAR2) enzyme catalyzes adenosine-to-inosine (A-to-I) RNA editing of mRNAs and microRNAs and controls brain development. However, the role of endothelial cell ADAR2 in vascular biology and inflammation has not been described so far.. Methods and Results: ADAR2 is expressed in human and murine endothelial cells and is 2-fold induced by hypoxia or hind limb ischemia in mice (P,0.05 for all). ADAR2 deficiency resulted in 73±12% impairment of leukocyte infiltration, in 53±4% reduced neovascularization, and a 40±6% decreased blood-flow recovery of ischemic muscle tissues in a hindlimb ischemia mouse model (P,0.001 for all). Mechanistically, among the highly ADAR2-regulated transcripts was interleukin-6 signal transducer (IL6ST or gp130), the receptor of interleukin-6 (IL-6). Silencing of ADAR2 resulted in a downregulation of gp130 mRNA and protein expression in endothelial cells by 65±5% and 50±5%, respectively (P,0.001 for both). Similarly, the ...
Introduction: Adenosine deaminase (ADA) is one of the major enzymes in purine metabolism. There are 2 isoforms of ADA: ADA1 and ADA2. The principal action of this enzyme is in immune system cells, the level of ADA in T-cell is 5-20 fold more than B-cell. The level of ADA elevates as the lymphocyte (T-cell) activity increase. Tuberculosis has been studied extensively with relevance of ADA levels and apart from serum, various body fluids as pleural, peritoneal, cerebrospinal fluids of patients of Pleural effusion, Ascitis and Tubercular Meningitis, has also its raised levels. Measurement of the level of (ADA) enzyme in body fluids is a helpful diagnostic tool. Aim: To study the serum Adenosine Deaminase Activity in patients of Pulmonary Tuberculosis and to evaluate the diagnostic significance of ADA activity in serum in these patients. Material and Methods: Present study was carried out in fifty patients of both the sexes with different ages suffering from Pulmonary Tuberculosis attending OPD and ...
The posttranscriptional modification of messenger RNA precursors (pre-mRNAs) by base deamination can profoundly alter the physiological function of the encoded proteins. The recent identification of tRNA-specific adenosine deaminases (ADATs) has led to the suggestion that these enzymes, as well as the cytidine and adenosine deaminases acting on pre-mRNAs (CDARs and ADARs), belong to a superfamily of RNA-dependent deaminases. This superfamily might have evolved from an ancient cytidine deaminase. This article reviews the reactions catalysed by these enzymes and discusses their evolutionary relationships.. ...
Adenosine deaminase RNA-specific B1 (ADARB1), an adenosine-to-inosine (A-to-I) RNA-editing enzyme, has been found to play an essential role in the development of cancer. However, the specific function of ADARB1 in lung cancer, especially in lung adenocarcinoma (LUAD), is still not fully understood and requires further study. In our study, integrative bioinformatics were used to analyze the detailed function of ADARB1 in LUAD. By conducting bioinformatics analyses of several public databases, such as Gene Expression Profiling Interactive Analysis (GEPIA), GE-mini, and Oncomine, we found significantly decreased ADARB1 expression in LUAD cells and tissues. Moreover, RT-PCR and Western blot showed lower ADARB1 expression in H358 and A549 LUAD cells compared to human bronchial epithelial Beas-2B cells. Wound Healing Assay indicated that knockdown ADARB1 could promote LUAD cell metastasis. By using the Kaplan-Meier Plotter tool, we found that downregulation of ADARB1 was related to shorter first ...
This is a clinical gene transfer study that aims to verify the safety and efficacy of the use of the EFS-ADA lentiviral vector to introduce the human adenosine deaminase (ADA) gene into the hematopoietic progenitors of patients affected with severe combined immunodeficiency due to ADA deficiency. The EFS-ADA vector expresses the human ADA cDNA under the control of the elongation factor alpha short promoter (EFS). In addition, this protocol will examine the effects of the ADA gene transfer on the immune system of treated patients. Patients with ADA deficiency and ineligible for matched sibling allogeneic bone marrow transplantation are eligible to participate in the study. To increase engraftment and selected advantage or gene-corrected cells, busulfan will be used as a cytoreductive agent. Enzyme replacement (PEG-ADA) will be discontinued 30 days after infusion of gene-corrected cells. CD34+ hematopoietic progenitors will be isolated from the patient bone marrow, peripheral blood or cord blood, ...
This study will evaluate a new method for delivering gene transfer therapy to patients with severe combined immunodeficiency disease (SCID) due to a defective adenosine deaminase (ADA) gene. This gene codes for the adenosine deaminase enzyme, which is essential for the proper growth and function of infection-fighting white blood cells called T and B lymphocytes. Patients who lack this enzyme are vulnerable to frequent and severe infections.. Some patients with this disease receive enzyme replacement therapy with weekly injections of the drug PEG-ADA (ADAGEN). This drug may increase the number of immune cells and reduce infections, but it is not a cure. Gene transfer therapy, in which a normal ADA gene is inserted into the patient s cells, attempts to correct the underlying cause of disease. This therapy has been tried in a small number of patients with varying degrees of success. In this study, the gene will be inserted into the patient s stem cells (cells produced by the bone marrow that mature ...
8-Azaadenosine is a potent ADAR1 (adenosine deaminases acting on double-stranded RNA) inhibitor. 8-Azaadenosine reduces A-to-I editing activity in a leukemia cell line, restores let-7 and inhibits leukemia stem cells self-renewal in vitro. - Mechanism of Action & Protocol.
OBJECTIVE: Adenosine deaminase (ADA) may be multifunctional, regulating adenosine levels and adenosine receptor (AR) agonism, and potentially modifying AR functionality. Herein we assess effects of ADA (and A(1)AR) deficiency on AR-mediated responses and ischaemic tolerance. METHODS: Normoxic function and responses to 20 or 25min ischaemia and 45min reperfusion were studied in isolated hearts from wild-type mice and from mice deficient in ADA and/or A(1)ARs. RESULTS: Neither ADA or A(1)AR deficiency significantly modified basal contractility, although ADA deficiency reduced resting heart rate (an effect abrogated by A(1)AR deficiency). Bradycardia and vasodilation in response to AR agonism (2-chloroadenosine) were unaltered by ADA deficiency, while A(1)AR deficiency eliminated the heart rate response. Adenosine efflux increased 10- to 20-fold with ADA deficiency (at the expense of inosine). Deletion of ADA improved outcome from 25min ischaemia, reducing ventricular diastolic pressure (by 45%; ...
... (ADA) is a protein produced by cells throughout the body and is associated with the activation of lymphocytes, a type of white blood cell that plays a role in the immune response to infections. The adenosine deaminase test may be used to help determine whether a person has a Mycobacterium tuberculosis infection (TB) of the lining of the lungs (pleurae).
We report the characterization of tadA, the first prokaryotic RNA editing enzyme to be identified. Escherichia coli tadA displays sequence similarity to the yeast tRNA deaminase subunit Tad2p. Recombinant tadA protein forms homodimers and is sufficient for site-specific inosine formation at the wobble position (position 34) of tRNA(Arg2), the only tRNA having this modification in prokaryotes. With the exception of yeast tRNA(Arg), no other eukaryotic tRNA substrates were found to be modified by tadA. How ever, an artificial yeast tRNA(Asp), which carries the anticodon loop of yeast tRNA(Arg), is bound and modified by tadA. Moreover, a tRNA(Arg2) minisubstrate containing the anticodon stem and loop is sufficient for specific deamination by tadA. We show that nucleotides at positions 33-36 are sufficient for inosine formation in mutant Arg2 minisubstrates. The anticodon is thus a major determinant for tadA substrate specificity. Finally, we show that tadA is an essential gene in E.coli, ...
Adenosine deaminase deficiency (also called ADA deficiency or ADA-SCID) is an autosomal recessive metabolic disorder that causes immunodeficiency. It occurs in fewer than one in 100,000 live births worldwide. It accounts for about 15% of all cases of severe combined immunodeficiency (SCID). ADA-SCID is a rare disease in which patients cannot make lymphocytes (a type of white blood cell) and, as a result, have a severely deficient immune system. A faulty gene inherited from both parents stops production of an essential protein called adenosine deaminase (ADA), which is particularly important for the formation of lymphocytes and a functioning immune system. Children born with ADA-SCID have an impaired ability to fight off everyday infections resulting in severe and life-threatening illness. They rarely survive beyond 1-2 years unless immune function is restored. Patients with ADA-SCID initially take antibiotics and antifungal treatments to help protect themselves from serious infections, but most ...
The treatment of SCID associated with ADA deficiency with ADAGEN® (pegademase bovine) Injection should be monitored by measuring plasma ADA activity and red blood cell dATP levels.. Plasma ADA activity and red cell dATP should be determined prior to treatment. Once treatment with ADAGEN® (pegademase bovine) Injection has been initiated, a desirable range of plasma ADA activity (trough level before maintenance injection) should be 15-35 μmol/hr/mL. This minimum trough level will ensure that plasma ADA activity from injection to injection is maintained above the level of total erythrocyte ADA activity in the blood of normal individuals.. Plasma ADA activity (pre-injection) should be determined every 1-2 weeks during the first 8-12 weeks of treatment in order to establish an effective dose of ADAGEN® (pegademase bovine) Injection. After 2 months of maintenance treatment with ADAGEN® (pegademase bovine) Injection, red cell dATP levels should decrease to a range of ≤ 0.005 to 0.015 μmol/mL. ...
Background The post-transcriptional processing of pre-mRNAs by RNA editing contributes significantly to the complexity of the mammalian transcriptome. RNA editing by site-selective A-to-I modification also regulates protein function through recoding of genomically specified sequences. The adenosine deaminase ADAR2 is the main enzyme responsible for recoding editing and loss of ADAR2 function in mice leads to a phenotype of epilepsy and premature death. Although A-to-I RNA editing is known to be subject to developmental and cell-type specific regulation, there is little knowledge regarding the mechanisms that regulate RNA editing in vivo. Therefore, the characterization of ADAR expression and identification of alternative ADAR variants is an important prerequisite for understanding the mechanisms for regulation of RNA editing and the causes for deregulation in disease. Methodology/Principal Findings Here we present evidence for a new ADAR2 splice variant that extends the open reading frame of ADAR2 by
A marked tissue-specific increase in erythrocyte adenosine deaminase (ADA) activity is associated with an autosomal dominantly inherited hemolytic anemia. We investigated the molecular basis of ADA overproduction by studying reticulocyte ADA mRNA from affected individuals. Analysis of proband reticulocyte ADA cDNA clones revealed normal sequence. RNase mapping demonstrated that the amount of ADA mRNA in affected reticulocytes was greater than the amount in normal B lymphoblasts, whereas ADA mRNA was undetectable in normal reticulocytes. The 5- and 3-untranslated regions of reticulocyte and B-lymphoblast ADA mRNAs from affected individuals were structurally indistinguishable from those of normal B lymphoblasts. Northern blot analysis performed under stringent hybridization and washing conditions confirmed a markedly increased amount of reticulocyte ADA mRNA in affected individuals as compared with controls. We conclude that the RBC-specific overexpression of ADA in this disorder occurs at the ...
Hereditary deficiency of the enzyme adenosie deaminase (adenosine aminohydrolase, EC 3.5.4.4) results in an immunodeficiency syndrome characterized by a marked reduction in circulating lymphocytes. We have administered 2-deoxycoformycin, a potent inhibitor of adenosine deaminase, to a patient with a lymphoproliferative malignancy. The clinical consequences of pharmacologic inhibition of adenosine deaminase activity included an abrupt decrease in the lymphocyte count, abnormalities of renal and hepatic function, and hemolytic anemia. The plasma concentrations of adenosine and deoxyadenosine rose to peak values of 13 microM and 5 microM, respectively, and erythrocyte dATP levels increased to 110 pmol/10(6) cells over 9 days. There was a corresponding decrease in erythrocyte ATP levels from 128 to , 6 pmol/10(6) cells. A similar profound reductin in ATP occurred in the erythrocytes of a second patient. The rapid and unexpected depletion of ATP associated with dATP accumulation may account, at ...
Adenosine deaminase (ADA) is a purine catabolic enzyme ubiquitous in mammalian tissue which catalyzes deamination of both adenosine and 2-deoxyadenosine to inosine and 2-deoxyinosine respectively. ...
Adenosin deaminase (ADA) deficiency is the cause for Severe Combined Immunodeficiency (SCID) in about 15% of patients with SCID, often presenting as T (-)B (-)NK (-)SCID. Treatment options for ADA-SCID are enzyme replacement, bone marrow transplantation or gene therapy. We here describe the first patient with ADA-SCID and fatal hepatic failure despite bone marrow transplantation from a 10/10 HLA identical related donor. As patients with ADA-SCID may be at yet underestimated increased risk for rapid hepatic failure we speculate whether hepatitis in ADA-SCID should lead to the immediate treatment with enzyme replacement by pegylated ADA. = Adenosindeaminasemangel (ADA-Mangel) ist bei 15% aller Patienten mit schwerem kombiniertem Immundefekt (SCID) ursächlich für die Erkrankung und präsentiert sich üblicherweise als T-B-NK-SCID. Behandlungsoptionen für ADA-Mangel sind Enzymersatztherapie, Knochenmarktransplantation und Gentherapie. Wir beschreiben hier den ersten Patienten mit ADA-SCID und ...
Klaus, Federica Rosina Patmina. Functional genetic variation of adenosine deaminase and the effects of sleep deprivation in healthy adults. 2010, University of Zurich, Faculty of Medicine. ...
Adenosine Deaminase 2/CECR1 Overexpression Lysate (Denatured). Tested Reactivity: Hu. Validated: WB. Backed by our 100% Guarantee.
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Has A-to-I RNA editing activity on extended dsRNA: edits RNA-binding protein Rnp4F. A-to-I editing of pre-mRNAs acts predominantly through nervous system targets to affect adult nervous system integrity, function and behavior. Essential for adaptation to environmental stresses, such as oxygen deprivation, and for the prevention of premature neuronal degeneration, through the editing of ion channels as targets.
Adarb1 - Lenti ORF clone of Adarb1 (Myc-DDK-tagged ORF) - Rat adenosine deaminase, RNA-specific, B1 (Adarb1), transcript variant 3, (10 ug) available for purchase from OriGene - Your Gene Company.
this is a serious disease that happens when your bodys defenses stop working because of a problem with your genes. you get ada-scid only if both your parents pass on a copy of a faulty gene to you.
Background and Aim: Diagnosis of tuberculous meningitis is difficult because of its non-specific clinical presentations which may be confused with other disorders of central nervous system. The initiation of anti-TB medication can often be delayed because of lack of available laboratory tests. This study was aimed at evaluating the adenosine ...
مجله علمی دانشگاه علوم پزشکی و خدمات درمانی بیرجند . داراي رتبه علمي- پژوهشي از كميسيون نشريات علوم پزشكي كشور Journal of Birjand University of Medical Sciencesfrom iran
The absence of an alternatively spliced exon between the two dsRBMs of dADAR in adult flies results in higher editing activity when compared to the isoform containing the exon seen at earlier stages of development. A number of constructs containing the dsRBMs were successfully cloned and purified. NMR data of RBM1 confirms that the dsRBMs of dADAR are members of the well characterised family of dsRBMs. Each dsRBM has a distinct affinity for dsRNA substrate indicating possible different roles. Point mutations within dsRBM1 result in a loss of binding activity. The self editing site of dADAR is found within exon 7 of the pre-mRNA, 3 to the second catalytic motif of the deaminase domain. Editing at this site results in a serine to glycine change in the translated protein and has been shown to reduce editing activity in all known substrates. This region of the protein is highly conserved throughout the ADARs and sequence comparisons with human ADAR2 at the DNA level show it has the potential to be ...
ADAR - ADAR (Myc-DDK-tagged)-Human adenosine deaminase, RNA-specific (ADAR), transcript variant 4 available for purchase from OriGene - Your Gene Company.
BioAssay record AID 1079594 submitted by ChEMBL: Selectivity ratio; ratio of pEC50 for human adenosine A2A receptor to pEC50 for human adenosine A3 receptor.
Hirschhorn R, Yang DR, Puck JM, Huie ML, Jiang CK, Kurlandsky LE, Spontaneous in vivo reversion to normal of an inherited mutation in a patient with adenosine deaminase deficiency [see comments] Nat Genet13:290-5 ...
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Psoriasis is a human skin condition characterized by epidermal hyperproliferation and infiltration of multiple leukocyte populations. Finally using a secreted and transmembrane protein library we found out high affinity relationships between human being IGFL1 and mIGFL and the TMEM149 ectodomain. TMEM149 (renamed here as IGFLR1) is an uncharacterized gene with structural similarity to the tumor necrosis element receptor family. Our studies demonstrate that IGFLR1 is definitely indicated primarily on the surface of mouse T cells. The connection between mIGFL and IGFLR1 receptor suggests mIGFL may influence T cell biology within inflammatory pores and skin conditions. (11). For hydrodynamic tail vein injection-induced manifestation of mIGFL 8 Balb/c mice were placed under a warmth light for 5 min before the injection to dilate the tail veins. Mice were then restrained in an acrylic chamber to allow access to their tails and 50 μg of bare pRK5 or pRK5 with N-terminal FLAG-tagged mIGFL inside a ...
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Principal Investigator:KOSHIBA Masahiro, Project Period (FY):2005 - 2006, Research Category:Grant-in-Aid for Scientific Research (C), Section:一般, Research Field:膠原病・アレルギー・感染症内科学
Supplementary Materials SUPPLEMENTARY DATA supp_42_13_e105__index. includes a well-calibrated type I mistake price using permuted ENCODE ChIP-seq data models; in contrast, two utilized gene established enrichment strategies frequently, Fishers exact ensure that you the binomial check applied in Genomic Locations Enrichment of Annotations Device (GREAT), can possess inflated type We mistake prices and biases in position […]. ...
Contact Us. Tel:732-484-9848. Fax:888-484-5008. Email:[email protected]. Add:1 Deer Park Dr, Suite Q,. Monmouth Junction, NJ 08852, USA. ...
The nucleoside, adenosine, is then deaminated and hydrolyzed to form hypoxanthine via adenosine deaminase and nucleosidase ... "Adenosine deaminase (ADA) deficiency". Learn.Genetics. Retrieved 31 October 2014. Nucleic Acids Book (free online book on the ... and adenosine deaminase deficiency, which causes immunodeficiency. Once the nucleotides are synthesized they can exchange ... Guanine is then deaminated via guanine deaminase to form xanthine which is then converted to uric acid. Oxygen is the final ...
APC Adenosine deaminase deficiency, partial; 102700; ADA Adenosine triphosphate, elevated, of erythrocytes; 102900; PKLR ...
When the enzyme adenosine deaminase is deficient in the body, the result is a toxic build-up of metabolites that impair ... ERT has also been used to treat patients with severe combined immunodeficiency (SCID) resulting from an adenosine deaminase ... Many ADA deficient children with SCID have been treated with the polyethylene glycol-conjugated adenosine deaminase (PEG-ADA) ... ERT has also been successful in treating severe combined immunodeficiency caused by an adenosine deaminase deficiency (ADA-SCID ...
"Entrez Gene: ADAR Adenosine Deaminase Acting on RNA". Samuel CE (2012). Adenosine deaminases acting on RNA (ADARs) and A-to-I ... which stands for adenosine deaminase acting on RNA). Adenosine deaminases acting on RNA (ADAR) are enzymes responsible for ... Adenosine Deaminase Acting on RNA is one of the most common forms of RNA editing, and has both selective and non-selective ... Adenosine Deaminase Acting on RNA (ADAR) and its gene were first discovered accidentally in 1987 as a result of research by ...
"Entrez Gene: USP40 ubiquitin specific peptidase 40". Bass BL (2002). "RNA editing by adenosine deaminases that act on RNA". ...
This gene encodes a member of a subfamily of the adenosine deaminase protein family. The encoded protein may act as a growth ... Zavialov AV, Engström A (2006). "Human ADA2 belongs to a new family of growth factors with adenosine deaminase activity". ... Charlab R, Valenzuela JG, Andersen J, Ribeiro JM (2001). "The invertebrate growth factor/CECR1 subfamily of adenosine deaminase ... factor and have adenosine deaminase activity. It may be responsible for some of the phenotypic features associated with cat eye ...
The use of an inhibitor of adenosine deaminase to increase the half-life of vidarabine has also been tried, and drugs such as ... It is prone to deamination by adenosine deaminase to inosine. This metabolite still possesses antiviral activity, but is 10- ... As you can see from figure 1.1 that it is a stereoisomer of adenosine. It has a half-life of 60 minutes, and its solubility is ... This is where ara-ATP is incorporated into the DNA strand replacing many of the adenosine bases. This results in the prevention ...
RNA-editing deaminase-2 (RED2, or ADARB2) is a member of the double-stranded RNA (dsRNA) adenosine deaminase family of RNA- ... "Entrez Gene: ADARB2 adenosine deaminase, RNA-specific, B2 (RED2 homolog rat)". Hong HQ, Lin JS, Chen L (Feb 2015). "Regulatory ... Chen CX, Cho DS, Wang Q, Lai F, Carter KC, Nishikura K (May 2000). "A third member of the RNA-specific adenosine deaminase gene ... Valenzuela A, Blanco J, Callebaut C, Jacotot E, Lluis C, Hovanessian AG, Franco R (Apr 1997). "Adenosine deaminase binding to ...
It is deaminated intracellularly by adenosine deaminase to dioxolane guanine (DXG). DXG-triphosphate, the active form of the ... Tenofovir is an acyclic adenosine derivative. The acyclic nature of the compound and its phosphonate moiety are unique ... First it is monophosphorylated by adenosine phosphotransferase and then the monophosphate is converted to carbovir 3´- ... In 1964 dideoxyadenosine, the corresponding adenosine analogue of zalcitabine was synthesised. Dideoxyadenosine caused kidney ...
"Entrez Gene: ADARB1 adenosine deaminase, RNA-specific, B1 (RED1 homolog rat)". Macbeth MR, Schubert HL, Vandemark AP, Lingam AT ... 1997). "Adenosine deaminase binding to human CD26 is inhibited by HIV-1 envelope glycoprotein gp120 and viral particles". J. ... 2000). "The HIV-1 gp120 inhibits the binding of adenosine deaminase to CD26 by a mechanism modulated by CD4 and CXCR4 ... Keegan LP, Leroy A, Sproul D, O'Connell MA (Feb 2004). "Adenosine deaminases acting on RNA (ADARs): RNA-editing enzymes". ...
... and within a million base pairs of the adenosine deaminase locus. It was also found to have an increase in expression in cells ... "Adenosine deaminase: characterization and expression of a gene with a remarkable promoter". EMBO J. 4 (2): 437-43. PMC 554205 ...
DPP-4 also binds the enzyme adenosine deaminase specifically and with high affinity. The significance of this interaction has ... Dipeptidyl peptidase-4 (DPP4), also known as adenosine deaminase complexing protein 2 or CD26 (cluster of differentiation 26) ... "Direct association of adenosine deaminase with a T cell activation antigen, CD26". Science. 261 (5120): 466-9. doi:10.1126/ ...
Note: adenosine is first metabolized to inosine via the enzyme adenosine deaminase. Nucleoside phosphorylase is an enzyme which ... PNPase, together with adenosine deaminase (ADA), serves a key role in purine catabolism, referred to as the salvage pathway. ... Adenosine uses the enzyme adenosine kinase, which is a very important enzyme in the cell. Attempts are being made to develop an ...
Bass B (2002). "RNA Editing by Adenosine Deaminases That Act on RNA". Annu Rev Biochem. 71: 817-46. doi:10.1146/annurev.biochem ... is most prevalent in higher eukaryotes converts adenosine nucleotides into inosine in dsRNAs via the enzyme adenosine deaminase ... Yang W, Wang Q, Howell K, Lee J, Cho D, Murray J, Nishikura K (2005). "ADAR1 RNA Deaminase Limits Short Interfering RNA ... "Modulation of microRNA processing and expression through RNA editing by ADAR deaminases". Nature Structural & Molecular Biology ...
Bass BL (2002). "RNA editing by adenosine deaminases that act on RNA". Annu. Rev. Biochem. 71: 817-46. doi:10.1146/annurev. ...
A to I RNA editing is catalyzed by a family of adenosine deaminases acting on RNA (ADARs) that specifically recognize ... Bass BL (2002). "RNA editing by adenosine deaminases that act on RNA". Annu. Rev. Biochem. 71: 817-46. doi:10.1146/annurev. ... The adenosine residue is mismatched in genomically encoded transcript, however this is not the case following editing. Despite ... Editing results in the targeted adenosine, which is mismatched prior to editing in the double-stranded RNA structure to become ...
D.K. Bhargava; M. Gupta; S. Nijhawan; S. Dasarathy; A.K.S. Kushwaha (June 1990). "Adenosine deaminase (ADA) in peritoneal ...
"About Center For Drug Design". Howard Schaeffer, S. Bittner, Robert Vince, S. Gurwara "Novel substrate of adenosine deaminase ...
Elo made her notable discovery of Adenosine Deaminase and Immune Deficiency in 1972. During the period when bone marrow ... Her numerous medical accomplishments include discovering the first immunodeficiency disease: adenosine deaminase deficiency. ...
These genes share close identity with the bacterial deaminases involved in nucleotide metabolism. The adenosine deaminase of E ... Carter, C.W. (1998). "Nucleoside deaminases for cytidine and adenosine: comparisons with deaminases acting on RNA". In: ... Adenosine deaminases acting on RNA (ADARs) are the RNA-editing enzymes involved in the hydrolytic deamination of Adenosine to ... The editing involves cytidine deaminase that deaminates a cytidine base into a uridine base. An example of C-to-U editing is ...
"Deoxyadenosine triphosphate acting as an energy-transferring molecule in adenosine deaminase inhibited human erythrocytes". ... Adenosine triphosphate (ATP) Romaniuk, P. J.; Eckstein, F. (1982-07-10). "A study of the mechanism of T4 DNA polymerase with ...
"Entrez Gene: AMPD3 adenosine monophosphate deaminase (isoform E)". "Peripheral blood lymphocytes data for Ampd3". Wellcome ... AMP deaminase 3 is an enzyme that in humans is encoded by the AMPD3 gene. This gene encodes a member of the AMP deaminase gene ... Yamada Y, Goto H, Wakamatsu N, Ogasawara N (2001). "A rare case of complete human erythrocyte AMP deaminase deficiency due to ... Yamada Y, Goto H, Murase T, Ogasawara N (1995). "Molecular basis for human erythrocyte AMP deaminase deficiency: screening for ...
Theoretically, this problem can be resolved if the effect of adenosine accumulated by EHNA, a result of adenosine deaminase ... "Adenosine deaminase inhibition enhances the inotropic response mediated by A1 adenosine receptor in hyperthyroid guinea pig ... EHNA is also a very potent adenosine deaminase inhibitor with an IC50 ~2 nM. This dual inhibition would lead to the ... Although EHNA potently inhibits adenosine deaminase, it has been successfully used with the proper controls as a tool to probe ...
... association with adenosine deaminase deficiency and non association with deoxyadenosine toxicity". Clinical Immunology and ... adenosine 5'-phosphatase, AMP phosphatase, adenosine monophosphatase, 5'-mononucleotidase, AMPase, UMPase, snake venom 5'- ... adenosine) which can readily enter most cells. Consequently, the enzyme plays a key role in the metabolism of nucleotides. The ... Nucleotidase in the Uptake of Adenosine from AMP by Human Lymphocytes" (PDF). Journal of Biological Chemistry. 250 (23): 8889- ...
Dipyridamole also inhibits the enzyme adenosine deaminase, the enzyme that catalyzes the breakdown of adenosine. Fenfluramine ... Dipyridamole inhibits reuptake of adenosine, resulting in greater extracellular concentrations of adenosine. ...
autosomal: Adenosine deaminase deficiency. *Omenn syndrome. *ZAP70 deficiency. *Bare lymphocyte syndrome. Acquired. *AIDS ...
Adenosine deaminase1/adensine deaminasep ratio improves the performance of adenosine deaminase activity in cases of false- ... Adenosine deaminase1/adenosine deaminasep correctly classified all nontuberculous lymphocytic pleural effusions with high ... Diagnostic value of adenosine deaminase in nontuberculous lymphocytic pleural effusions. D. Jiménez Castro, G. Díaz Nuevo, E. ... Diagnostic value of adenosine deaminase in nontuberculous lymphocytic pleural effusions. D. Jiménez Castro, G. Díaz Nuevo, E. ...
AMPD2; adenosine monophosphate deaminase 2; adenosine monophosphate deaminase 2 (isoform L); AMP deaminase 2; AMPD isoform L; ... adenosine monophosphate deaminase 2 isoform L; AMP deaminase isoform L; AMPD 2; AMPD2_HUMAN; AMPD ... Adenosine monophosphate deaminase-2 (EC 3.5.4.6) catalyzes the deamination of AMP to IMP and plays an important role in the ...
Estimation of serum Adenosine Deaminase (ADA) level in sickle cell disease (SCD) and its association with reticulocyte count in ... Adenosine deaminase level in SCD was higher as compare to control group and difference is statistically highly significant i.e ... Estimation of serum Adenosine Deaminase (ADA) level in sickle cell disease (SCD) and its association with reticulocyte count in ... Estimation of Adenosine Deaminase level can be helpful for knowing pathophysiology of complications occurring in sickle cell ...
T1 - Conversion of a stem cell leukemia from a T-lymphoid to a myeloid phenotype induced by the adenosine deaminase inhibitor 2 ... Conversion of a stem cell leukemia from a T-lymphoid to a myeloid phenotype induced by the adenosine deaminase inhibitor 2- ... Conversion of a stem cell leukemia from a T-lymphoid to a myeloid phenotype induced by the adenosine deaminase inhibitor 2- ... Conversion of a stem cell leukemia from a T-lymphoid to a myeloid phenotype induced by the adenosine deaminase inhibitor 2- ...
Myoadenylate deaminase catalyses the reaction from adenosine monophosphate (AMP) to inosine monophosphate (IMP) plus ammonia ( ... Hisatome, I., Morisake, T., Kamma, H., Sugama, T., Morisaki, H., Ohtahara, A. & Holmes, E. W. (1998). Control of AMP deaminase ... Shumate J. B., Katnik, R., Ruiz, M., Kaiser, K., Frieden, C., Brooke, M. H. & Carroll, J. E. (1979). Myoadenylate deaminase ... Sinkeler S. P., Binkhorst, R. A., Joosten, E. M., Wevers, R. A., Coerwinkei, M. M. & Oei, T. L. (1987). AMP deaminase ...
In the noninvasive tests, both the ascitic adenosine deaminase (ADA) level over 30 U/l, and the ascitic lactate dehydrogenase ( ... In the noninvasive tests, both the ascitic adenosine deaminase (ADA) level over 30 U/l, and the ascitic lactate dehydrogenase ( ... In the noninvasive tests, both the ascitic adenosine deaminase (ADA) level over 30 U/l, and the ascitic lactate dehydrogenase ( ... In the noninvasive tests, both the ascitic adenosine deaminase (ADA) level over 30 U/l, and the ascitic lactate dehydrogenase ( ...
Because only small amounts of adenosine deaminase are necessary for competent immunity, some patients with ADA mutations may ... adenosine deaminase activity and no profound immunodeficiency. [2, 3] Epidemiology Frequency United States PNP [emedicine.com] ... 4, 5, 6, 7] In the preceding step of the pathway, ADA metabolizes adenosine to inosine. [emedicine.com] ...
... information resources and questions answered by our Genetic and Rare Diseases Information Specialists for Adenosine deaminase ... Adenosine deaminase deficiency Title Other Names:. ADA deficiency; Severe combined immunodeficiency due to adenosine deaminase ... due to adenosine deaminase deficiency; ADA-SCID; Adenosine deaminase deficient severe combined immunodeficiency See More ... ghr.nlm.nih.gov/condition/adenosine-deaminase-deficiency. *Hershfield M. Adenosine Deaminase Deficiency. GeneReviews. Mar 2017 ...
Coformycin was also described as a adenosine deaminase inhibitor but is alleged to be an antibiotic. Adenosine deaminase ... Adenosine deaminase (also known as adenosine aminohydrolase, or ADA) is an enzyme (EC 3.5.4.4) involved in purine metabolism. ... Adenosine deaminase deficiency leads to pulmonary fibrosis, suggesting that chronic exposure to high levels of adenosine can ... Blackburn MR (2003). "Too much of a good thing: adenosine overload in adenosine-deaminase-deficient mice". Trends in ...
Other names in common use include adenylate deaminase, adenine nucleotide deaminase, and adenosine (phosphate) deaminase. Su JC ... In enzymology, an adenosine-phosphate deaminase (EC 3.5.4.17) is an enzyme that catalyzes the chemical reaction 5-AMP + H2O ... Yates MG (1969). "A non-specific adenine nucleotide deaminase from desulfovibrio desulfuricans". Biochim. Biophys. Acta. 171 (2 ... The systematic name of this enzyme class is adenosine-phosphate aminohydrolase. ...
The adenosine deaminase test may be used to help determine whether a person has a Mycobacterium tuberculosis infection (TB) of ... Adenosine deaminase (ADA) is a protein produced by cells throughout the body and is associated with the activation of ... The adenosine deaminase (ADA) test is not a diagnostic test, but it may be used along with other tests such as pleural fluid ... Adenosine deaminase (ADA) is a protein that is produced by cells throughout the body and is associated with the activation of ...
... medlineplus.gov/genetics/condition/adenosine-deaminase-deficiency/ Adenosine deaminase deficiency. ... mediated immunosuppression and the role of adenosine in causing the immunodeficiency associated with adenosine deaminase ... Adenosine deaminase deficiency is caused by mutations in the ADA gene. This gene provides instructions for producing the enzyme ... Adenosine deaminase deficiency is very rare and is estimated to occur in approximately 1 in 200,000 to 1,000,000 newborns ...
The adenosine deaminase (ADA) test is not a diagnostic test, but it may be used along with other tests such as pleural fluid ... Adenosine deaminase (ADA) is a protein that is produced by cells throughout the body and is associated with the activation of ... If adenosine deaminase (ADA) is markedly elevated in pleural fluid in a person with signs and symptoms that suggest ... 2010 October). Adenosine Deaminase Levels in CSF of Tuberculous Meningitis Patients. J Clin Med Res. 2010 October; 2(5): 220- ...
... ,ARUP Laboratories is a national reference laboratory and a worldwide leader in innovative laboratory ...
How is adenosine deaminase severe combined immunodeficiency (ADA-SCID) treated?. *What are treatments for adenosine deaminase ... What causes adenosine deaminase severe combined immunodeficiency (ADA-SCID)?. ANSWER This is a serious disease that happens ... How do you take care of yourself or your child with adenosine deaminase severe combined immunodeficiency (ADA-SCID)? ... What do you need to know about adenosine deaminase severe combined immunodeficiency (ADA-SCID)? ...
The adenosine deaminase (ADA) metabolism. ADA is an enzyme of the purine salvage pathway, which catalyzes the irreversible ... Autoimmune dysregulation and purine metabolism in adenosine deaminase deficiency.. Sauer AV1, Brigida I, Carriglio N, Aiuti A. ... Genetic defects in the adenosine deaminase (ADA) gene are among the most common causes for severe combined immunodeficiency ( ... PEG-ADA present in the extracellular space eliminates adenosine produced by the ectoenzymatic chain and hinders adenosine- ...
... adenosine aminohydrolase, ADA, EC 3.5.4.4.) is widely distributed in human tissues. In some tissues ADA exists exclusively as ... Adenosine deaminase (adenosine aminohydrolase, ADA, EC 3.5.4.4.) is widely distributed in human tissues. In some tissues ADA ... Adenosine Deaminase Large Form Small Form Radioactive Peak Native Molecular Weight These keywords were added by machine and not ... W.P. Schrader and A.R. Stacy, Purification and subunit structure of adenosine deaminase from human kidney. J. Biol. Chem. 252: ...
Here, through analysis of genome-scale loss-of-function datasets, we identify adenosine deaminase acting on RNA (ADAR or ADAR1 ... Identification of ADAR1 adenosine deaminase dependency in a subset of cancer cells. *Hugh S. Gannon1,2. na1, ... Gannon, H.S., Zou, T., Kiessling, M.K. et al. Identification of ADAR1 adenosine deaminase dependency in a subset of cancer ... Pfaller, C. K., Li, Z., George, C. X. & Samuel, C. E. Protein kinase PKR and RNA adenosine deaminase ADAR1: new roles for old ...
Adenosine deaminase, commonly used biomarker for the diagnosis, is non specific and there is paucity of literature on its... ... Barua R, Hossain M. Adenosine deaminase in diagnosis of tuberculosis: a review. Anwer Khan Mod Med Coll J. 2014;5:43-8.CrossRef ... Adenosine deaminase, commonly used biomarker for the diagnosis, is non specific and there is paucity of literature on its ... Diagnostic value of adenosine deaminase in tuberculous and malignant pleural effusion. Egypt J Chest Dis Tuberc. 2012;61(4):413 ...
Keywords: adenosine deaminase; interferon type II; laboratory techniques; meta-analysis; procedure; tuberculous pleurisy ... Adenosine deaminase and interferon gamma measurements for the diagnosis of tuberculous pleurisy: a meta-analysis ... several biochemical and immunological markers have been proposed to diagnose tuberculous pleurisy including adenosine deaminase ...
... inhibits adenosine deaminase activity and reduces glucose levels in hyperglycemic patients. ... Adenosine deaminase (ADA) is an important enzyme that plays a relevant role in purine and DNA metabolism, immune responses, and ... Pharmacological Actions : Adenosine deaminase inhibitor : CK(16) : AC(5), Antioxidants : CK(14410) : AC(5758), Hypoglycemic ... Syzygium cumini inhibits adenosine deaminase activity and reduces glucose levels in hyperglycemic patients. ...
Compare adenosine deaminase, RNA specific B2 (inactive) ELISA Kits from leading suppliers on Biocompare. View specifications, ... adenosine deaminase, RNA specific B2 (inactive) ELISA Kits. The ELISA (enzyme-linked immunosorbent assay) is a widely used ...
... is used to treat adenosine deaminase severe combined immune deficiency (ADA-SCID). Includes Revcovi side effects, interactions ... Revcovi (elapegademase-lvlr) is a recombinant adenosine deaminase indicated for the treatment of adenosine deaminase severe ... mutations in the ADA gene reduce or eliminate the protective activity of adenosine deaminase, allowing the buildup of adenosine ... FDA Approves Revcovi (elapegademase-lvlr) for Adenosine Deaminase Severe Combined Immune Deficiency (ADA-SCID) ...
Adenosine and AMP deaminases family. Adenosine deaminase subfamily.UniRule annotation. Manual assertion according to rulesi ... IPR001365 A/AMP_deaminase_dom. IPR028893 A_deaminase. IPR006330 Ado/ade_deaminase. IPR032466 Metal_Hydrolase. ... IPR001365 A/AMP_deaminase_dom. IPR028893 A_deaminase. IPR006330 Ado/ade_deaminase. IPR032466 Metal_Hydrolase. ... sp,A5U7Y8,ADD_MYCTA Adenosine deaminase OS=Mycobacterium tuberculosis (strain ATCC 25177 / H37Ra) OX=419947 GN=add PE=3 SV=1 ...
Heat-induced formation of alpha,beta-unsaturated nucleoside dialdehydes and their activity with adenosine deaminase.. Grant AJ ... The reaction was first discovered during studies with adenosine deaminase and was initially investigated enzymatically until ... Adenosine dialdehyde, obtained by periodate oxidation of adenosine, afforded the same product upon heating as obtained by ...
  • BACKGROUND--A statistical audit of adenosine deaminase (ADA) in pleural effusions was undertaken. (bmj.com)
  • Adenosine deaminase (ADA) can aid in the diagnosis of tuberculous pleural effusions, but false-positive findings from lymphocytic effusions have been reported. (ersjournals.com)
  • This prospective study provides additional evidence that adenosine deaminase levels in nontuberculous lymphocytic pleural effusions seldom exceed the cut-off set for tuberculous effusions. (ersjournals.com)
  • Adenosine deaminase 1 /adenosine deaminase p correctly classified all nontuberculous lymphocytic pleural effusions with high adenosine deaminase levels. (ersjournals.com)
  • For decades, physicians, patients, and their families have relied upon enzyme replacement therapy as a life-saving treatment for adenosine deaminase severe combined immunodeficiency, a disease in which the buildup of toxic metabolites can cripple children's immune systems," said Morna Dorsey, M.D., MMSc, Professor of Pediatrics at the University of California, San Francisco. (drugs.com)
  • Gene transfer into autologous hematopoietic stem cells by γ-retroviral vectors (gRV) is an effective treatment for adenosine deaminase (ADA)-deficient severe combined immunodeficiency (SCID). (iupui.edu)
  • Western Blot: Adenosine Deaminase/ADA Antibody [NBP1-- Lane 1: Adenosine Deaminase (Calf Spleen). (novusbio.com)
  • Western Blot: Adenosine Deaminase/ADA Antibody [NBP1-- Used to detect adenosine deaminase in mouse pancreas lysate (Left, Lane 1, 30 ul) under reducing conditions. (novusbio.com)
  • Adenosine Deaminase antibody LS-C3557 is an unconjugated rabbit polyclonal antibody to Adenosine Deaminase (ADA) from human, mouse, rat and other species. (lsbio.com)
  • MBS2703267 is a ready-to-use microwell, strip plate Double-antibody Sandwich ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Adenosine Deaminase (ADA) ELISA Kit target analytes in biological samples. (mybiosource.com)
  • The microtiter plate provided in this kit has been pre-coated with an antibody specific to Adenosine Deaminase (ADA). (mybiosource.com)
  • Standards or samples are then added to the appropriate microtiter plate wells with a biotin-conjugated antibody specific to Adenosine Deaminase (ADA). (mybiosource.com)
  • After TMB substrate solution is added, only those wells that contain Adenosine Deaminase (ADA), biotin-conjugated antibody and enzyme-conjugated Avidin will exhibit a change in color. (mybiosource.com)
  • Identification of ADAR1 adenosine deaminase depend. (cancer.gov)
  • The adenosine deaminase (ADA) test is not a diagnostic test, but it may be used along with other tests such as pleural fluid analysis , acid-fast bacillus (AFB) smear and culture , and/or tuberculosis molecular testing to help determine whether a person has a Mycobacterium tuberculosis infection (tuberculosis or TB) of the lining of the lungs (pleurae). (labcorp.com)
  • This study was set up to investigate the diagnostic value of serum Adenosine deaminase in diagnosis of tuberculosis. (semanticscholar.org)
  • The present study evaluates interleukin-6 (IL-6), gamma interferon (IFN- γ ) and adenosine deaminase (ADA) as diagnostic tools in pleural effusion. (scielo.org)
  • The serendipitous discovery of adenosine deaminase (ADA) deficiency in two patients with cellular immunodeficiency in 1972 by Dr. Eloise Giblett and colleagues ( 1 ) ushered in a new era in the investigation of the molecular mechanisms underlying primary immunodeficiency disorders. (jimmunol.org)
  • Pillars Article: Adenosine-Deaminase Deficiency in Two Patients with Severely Impaired Cellular Immunity. (jimmunol.org)
  • This test measures the amount of adenosine deaminase present in pleural fluid in order to help diagnose a tuberculosis infection of the pleurae . (labtestsonline.org)
  • Las neoplasias y la tuberculosis son las causas más frecuentes en los diagnósticos de tales derrames. (scielo.org)
  • Los métodos de laboratorio convencionales para el diagnóstico de tales derrames son ineficientes, porque los bacilos de la tuberculosis raramente se ven en los exámenes directos del líquido pleural. (scielo.org)
  • In enzymology, an adenosine-phosphate deaminase (EC 3.5.4.17) is an enzyme that catalyzes the chemical reaction 5'-AMP + H2O ⇌ {\displaystyle \rightleftharpoons } 5'-IMP + NH3 Thus, the two substrates of this enzyme are 5'-AMP and H2O, whereas its two products are 5'-IMP and NH3. (wikipedia.org)
  • GAITHERSBURG, Md.--(BUSINESS WIRE) October 05, 2018 --Leadiant Biosciences, Inc. today announced that the Food and Drug Administration (FDA) has granted approval to Revcovi™ (elapegademase-lvlr) injection in the U.S. Revcovi is a new enzyme replacement therapy (ERT) for the treatment of adenosine deaminase severe combined immune deficiency (ADA-SCID) in pediatric and adult patients. (drugs.com)
  • By providing specific and direct replacement of the adenosine deaminase enzyme, Revcovi can reduce patients' risk of potentially serious, life-threatening infections and their debilitating complications. (drugs.com)
  • This study will monitor the long-term effects of gene therapy in patients with severe combined immunodeficiency disease (SCID) due to a deficiency in an enzyme called adenosine deaminase (ADA). (clinicaltrials.gov)
  • This study will evaluate a new method for delivering gene transfer therapy to patients with severe combined immunodeficiency disease (SCID) due to a defective adenosine deaminase (ADA) gene. (clinicaltrials.gov)
  • Adenosin deaminase (ADA) deficiency is the cause for Severe Combined Immunodeficiency (SCID) in about 15% of patients with SCID, often presenting as T (-)B (-)NK (-)SCID. (uzh.ch)
  • We previously reported that the elevated adenosine deaminase (ADA) activities in the joints of rheumatoid arthritis (RA) patients contribute to the pathogenesis of RA by neutralizing the anti-rheumatic properties of endogenous adenosine. (nii.ac.jp)
  • Many cases of severe combined immunodeficiency syndrome in humans result from a heritable lack of adenosine deaminase. (thefreedictionary.com)
  • Here, through analysis of genome-scale loss-of-function datasets, we identify adenosine deaminase acting on RNA ( ADAR or ADAR1) as an essential gene for the survival of a subset of cancer cell lines. (nature.com)
  • We find that lung cancer cell lines expressing high levels of interferon-stimulated genes (ISGs) are vulnerable to deletion of the RNA adenosine deaminase, ADAR or ADAR1. (nature.com)
  • Three days later, an adenosine deaminase (ADA) level of 118.1 U/L (normal range, 0.0-11.3 U/L) from pericardial fluid was reported from the laboratory. (ama-assn.org)
  • This gene encodes a Drosophila pre-mRNA adenosine deaminase (dADAR) and is expressed almost exclusively in the adult central nervous system. (jci.org)