DeficiencyEnzymeReceptorsInhibitorIntracellularTriphosphateProteinADARExtracellular adenosinePhosphorylationDiphosphateNucleoside transportersConverts adenosineDeamination of adenosinePurinePyruvate KinaseAdenylate KinaseDipyridamoleInhibitsDecreasesEndogenous nucleosideTransportersGlutamateNucleotideUric acidRapidlyInhibitionAccumulationInflammationEnzymesHepaticLactateIsoformGeneIncreasesMechanismDeoxyadenosinePathwayBolus
Deficiency18
- Adenosine monophosphate deaminase deficiency type 1 or AMPD1, is a human metabolic disorder in which the body consistently lacks the enzyme AMP deaminase, in sufficient quantities. (wikipedia.org)
- The disease was formerly known as myoadenylate deaminase deficiency (MADD). (wikipedia.org)
- citation needed] AMPD1 deficiency is caused by a defect in the mechanism for production of AMP deaminase - an enzyme that converts adenosine monophosphate (AMP) to inosine monophosphate (IMP). (wikipedia.org)
- Purine nucleoside phosphorylase is a ubiquitously expressed enzyme of the purine salvage pathway as is adenosine deaminase, deficiency of which also causes immunodeficiency. (frontiersin.org)
- PNP deficiency results in the accumulation of deoxyguanosine, which is converted to deoxyguanosine triphosphate (dGTP) by deoxycytidine kinase specific to lymphocytes. (frontiersin.org)
- Kohn's name may be familiar, as he has devoted his career to developing gene therapy for PIs, including adenosine deaminase deficient severe combined immunodeficiency (ADA-SCID ) and leukocyte adhesion deficiency-I (LAD-1) . (primaryimmune.org)
- Adenosine deaminase (ADA) deficiency is an inherited disorder that damages the immune system and causes severe combined immunodeficiency (SCID). (nih.gov)
- Adenosine deaminase 2 (ADA2) deficiency is a disorder characterized by abnormal inflammation of various tissues. (nih.gov)
- Adenosine monophosphate (AMP) deaminase deficiency is a condition that can affect the muscles used for movement (skeletal muscles). (nih.gov)
- Adenosine deaminase (ADA) deficiency accounts for about half of the autosomal recessive forms of SCIDs. (lu.se)
- Mevalonate kinase deficiency (aka Hyper IgD syndrome). (clevelandclinic.org)
- INTRODUCTION - Adenosine deaminase (ADA) deficiency (MIM #102700) was the first immunodeficiency in which the specific molecular defect was identified. (medilib.ir)
- See "Adenosine deaminase deficiency: Treatment and prognosis" and "Purine nucleoside phosphorylase deficiency" . (medilib.ir)
- Treatment of myoadenylate deaminase deficiency is exercise modulation as appropriate. (msdmanuals.com)
- Diagnosis of adenosine deaminase deficiency is by DNA analysis. (msdmanuals.com)
- Treatment of adenosine deaminase deficiency is by bone marrow or stem cell transplantation and enzyme replacement therapy. (msdmanuals.com)
- Cladribine and its rage of energy Carson et al 50, 51 discfromed that the lymphopenia observed in an inherited disstraighten out of adenosine deaminase deficiency was caworn not later than the accumulation of deoxyadenosine nucleotides within lymphocytes. (upb.ro)
- Pyruvate kinase deficiency is an inherited red blood cell enzyme disorder that causes chronic hemolysis. (conduent.com)
Enzyme12
- AMP deaminase is an enzyme that converts adenosine monophosphate (AMP) to inosine monophosphate (IMP), freeing an ammonia molecule in the process. (wikipedia.org)
- This gene encodes the enzyme responsible for pre-mRNA editing of the glutamate receptor subunit B by site-specific deamination of adenosines. (cancerindex.org)
- Adenosine deamination and the ADAR enzyme family. (biomedcentral.com)
- ADA gene provides instructions for producing the enzyme adenosine deaminase. (nih.gov)
- gene provides instructions for making an enzyme called adenosine deaminase 2. (nih.gov)
- This enzyme breaks down molecules called adenosine and 2'-deoxyadenosine. (nih.gov)
- gene provides instructions for producing an enzyme called adenosine monophosphate (AMP) deaminase. (nih.gov)
- ADA2 is another enzyme that has partial structural homology with ADA1 and is able to convert adenosine and 2-deoxyadenosine, albeit at a much lower affinity. (medilib.ir)
- Since adenosine has antinociceptive effects in rodents and humans, we hypothesized that NT5E, an enzyme that generates adenosine, might also have antinociceptive effects in vivo . (biomedcentral.com)
- The enzyme myoadenylate deaminase converts AMP to inosine and ammonia. (msdmanuals.com)
- Nucleoside diphosphate kinase is the primary enzyme involved in phosphorylation of the diphosphate to the active triphosphate, PSI-352666. (inrae.fr)
- Affected individuals are either homozygous for a single pathogenic mutation or compound heterozygous for two different pathogenic variants affecting the function of the pyruvate kinase enzyme in red blood cells and liver. (conduent.com)
Receptors13
- Adenosine mediates pain through adenosine receptors. (wikipedia.org)
- Over time, excess free adenosine down-regulates primary A1 adenosine receptors, leading to increased muscle pain. (wikipedia.org)
- Adenosine is thought to exert its pharmacological effects through activation of purine receptors (cell-surface A1 and A2 adenosine receptors). (globalrph.com)
- Although the exact mechanism by which adenosine receptor activation relaxes vascular smooth muscle is not known, there is evidence to support both inhibition of the slow inward calcium current reducing calcium uptake, and activation of adenylate cyclase through A2 receptors in smooth muscle cells. (globalrph.com)
- Adenosine receptors are closely associated with dopamine receptors and form heteromer s with them. (adxs.org)
- At the same time, adenosine modulates striatal DA release by stimulating glutamate release at adenosine receptors in the striatum , which increases dopamine levels. (adxs.org)
- Part of this adenosine is discharged from the cell and binds to adenosine receptors of neighboring cells, which is supposed to compensate for the disturbed balance between energy consumption and energy supply. (adxs.org)
- Cellular signalling occurs through adenosine receptors (ARs). (turkupetcentre.net)
- All four adenosine receptor subtypes (A 1 , A 2A , A 2B , and A 3 ) are G protein-coupled receptors (GPCRs), either increasing or decreasing intracellular cAMP levels by affecting adenylate cyclase activity. (turkupetcentre.net)
- Like other GPCRs, adenosine receptors have a single polypeptide chain, forming three extracellular and three intracellular loops. (turkupetcentre.net)
- Adenosine receptors are also referred to as purine receptors , or P1 purinoceptors . (turkupetcentre.net)
- Adenosine has the highest affinity to the A 1 R and A 2A R, intermediate affinity to A 3 R, and lowest affinity to A 2B R. Adenosine activates A 1 and A 2A receptors already in nanomolar concentrations. (turkupetcentre.net)
- We conclude that hypertonic stress regulates neutrophil function via a single molecule (ATP) and its metabolite (adenosine), using positive- and negative-feedback mechanisms through the activation of P2 and A2 receptors, respectively. (escholarship.org)
Inhibitor5
- ABT-702 (4-amino-5-(3-bromophenyl)-7-(6-morpholinopyridin-3-yl)pyrido[2, 3-d]pyrimidine), a novel orally effective adenosine kinase inhibitor with analgesic and anti-inflammatory. (axonmedchem.com)
- Discovery of 4-amino-5-(3-bromophenyl)-7-(6-morpholino-pyridin-3-yl)pyrido[2,3-d]pyrimidine, an orally active, non-nucleoside adenosine kinase inhibitor. (axonmedchem.com)
- extract could inhibit the development of Lewis GSK2126458 small molecule kinase inhibitor lung, A549 and SBC-3 cancers cells, and induce cancers cell apoptosis (23). (insulin-receptor.info)
- However, it GSK2126458 small molecule kinase inhibitor remains unclear whether ILL can inhibit lung malignancy cell metastasis and the associated mechanisms require further investigation. (insulin-receptor.info)
- Alpelisib is small-molecule inhibitor of phosphatidylinositol-3 kinase (PI3K). (conduent.com)
Intracellular5
- Intracellular adenosine is rapidly metabolized either via phosphorylation to adenosine monophosphate by adenosine kinase, or via deamination to inosine by adenosine deaminase in the cytosol. (nih.gov)
- The intracellular uptake of adenosine is mediated by a specific transmembrane nucleoside transport system. (globalrph.com)
- These intracellular metabolites of adenosine are not vasoactive. (globalrph.com)
- Intracellular adenosine production and release is tightly regulated, and it is not stored in vesicles. (turkupetcentre.net)
- MTX increases intracellular accumulation of adenosine monophosphate (AMP) and 5-aminoimidazole-4-carboxamide ribonucleotide which activates AMP-activated protein kinase (AMPK). (bmj.com)
Triphosphate8
- In order to use energy, a cell converts one of the above fuels into adenosine triphosphate (ATP) via the mitochondria. (wikipedia.org)
- It is formed either inside or on the surface of cells via the breakdown of nucleotides (the basic building blocks of DNA and RNA) or adenine phosphates: energy-rich adenosine triphosphate (ATP), adenosine diphosphate (ADP), and adenosine monophosphate (AMP). (selfhacked.com)
- Adenosine triphosphate or ATP is known as the body's "energy currency. (selfhacked.com)
- cell's main energy source, a molecule called adenosine triphosphate (ATP). (nih.gov)
- a component of adenosine triphosphate and adenosine diphosphate. (adxs.org)
- It has a central role in cell metabolism and signalling as such and as the backbone for ATP (adenosine triphosphate) and cAMP (cyclic adenosine monophosphate). (turkupetcentre.net)
- Adenosine and deoxyadenosine are also converted to 5'-deoxyadenosine triphosphate (dATP), which inhibits ribonucleotide reductase and prevents de novo synthesis of nucleotides and deoxynucleotides. (medilib.ir)
- Here, we report that HS dose-dependently releases adenosine 5'-triphosphate (ATP) from neutrophils and that extracellular ATP is rapidly converted to adenosine or activates p38 MAPK and enhances N-formyl-methionyl-lencyl-phenylalanine-induced superoxide formation. (escholarship.org)
Protein7
- ADAR deletion causes activation of the double-stranded RNA sensor, protein kinase R (PKR). (nih.gov)
- instructions for making a protein called RNA-specific adenosine deaminase 1 (ADAR1). (nih.gov)
- Ecto-5'-nucleotidase (NT5E) is a glycosyl phosphatidylinositol (GPI)-anchored membrane protein that catalyzes the hydrolysis of extracellular AMP to adenosine [ 1 ]. (biomedcentral.com)
- These data suggested that spinal delivery of PAP protein could be used therapeutically to generate adenosine and activate A 1 R over an extended time period. (biomedcentral.com)
- Considering that both PAP and NT5E generate adenosine, we hypothesized that NT5E protein might also have A 1 R-dependent antinociceptive effects. (biomedcentral.com)
- Histidine triad nucleotide-binding protein 1 (Hint 1) then removes the amino acid moiety, which is followed by hydrolysis of the methoxyl group at the O(6)-position of the guanine base by adenosine deaminase-like protein 1 (ADAL1) to give 2'-deoxy-2'-fluoro-2'-C-methylguanosine-5'-monophosphate. (inrae.fr)
- We have previously shown that hypertonic stress (HS) can suppress chemoattractant-induced neutrophil responses via cyclic adenosine monophosphate and enhance these responses through p38 mitogen-activated protein kinase (MAPK) activation. (escholarship.org)
ADAR4
- 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. (nih.gov)
- It is catalyzed by ADAR (adenosine deaminase acting on RNA) enzymes, which exist throughout the body but are most prevalent in the central nervous system. (biomedcentral.com)
- A-to-I editing, which is catalyzed by enzymes of the adenosine deaminase acting on RNA (ADAR) family, is most prevalent in the central nervous system (CNS) but occurs in many tissues [ 1 - 3 ]. (biomedcentral.com)
- ADAR enzymes catalyze the A-to-I hydrolytic deamination reaction, by which an adenosine loses an amine group and is converted to inosine. (biomedcentral.com)
Extracellular adenosine4
- While extracellular adenosine is primarily cleared by cellular uptake with a half-life of less than 10 seconds in whole blood, excessive amounts may be deaminated by an ecto-form of adenosine deaminase. (nih.gov)
- its production, both intracellularly and extracellularly, is tightly coupled to energy consumption resulting in increasing extracellular adenosine levels with increased energy consumption. (dissertations.se)
- Dipyridamole elevates extracellular adenosine concentrations by inhibiting equilibrative nucleoside transporters (ENTs). (turkupetcentre.net)
- Ecto-5'-nucleotidase (NT5E, also known as CD73) hydrolyzes extracellular adenosine 5'-monophosphate (AMP) to adenosine in nociceptive circuits. (biomedcentral.com)
Phosphorylation3
- Since adenosine kinase has a lower K m and V max than adenosine deaminase, deamination plays a significant role only when cytosolic adenosine saturates the phosphorylation pathway. (nih.gov)
- Adenosine monophosphate formed by phosphorylation of adenosine is incorporated into the high-energy phosphate pool. (nih.gov)
- If Hog1's stepivation in these procrastinates is achieved during going of autophosphorylation, the kinase-dead mutation should abolish its phosphorylation. (upb.ro)
Diphosphate2
- Cellular processes, especially muscles, then convert the ATP into adenosine diphosphate (ADP), freeing the energy to do work. (wikipedia.org)
- The monophosphate is phosphorylated to the diphosphate by guanylate kinase. (inrae.fr)
Nucleoside transporters1
- Adenosine cannot freely pass across the cell and vesicular membranes, but it needs to be transported by nucleoside transporters, which are present in most cells, including erythrocytes . (turkupetcentre.net)
Converts adenosine2
- Adenosine kinase in red blood cells converts adenosine into adenine nucleotides. (turkupetcentre.net)
- Adenosine deaminase converts adenosine and deoxyadenosine to inosine and deoxyinosine, which are further broken down and excreted. (msdmanuals.com)
Deamination of adenosine2
- Inosine formed by deamination of adenosine can leave the cell intact or can be degraded to hypoxanthine, xanthine, and ultimately uric acid. (nih.gov)
- It catalyzes the deamination of adenosine and deoxyadenosine to inosine and deoxyinosine, which are converted to waste products and excreted. (medilib.ir)
Purine5
- PNP, with adenosine deaminase (ADA), is part of the purine salvage pathway. (frontiersin.org)
- As a nucleoside, adenosine is made of an adenine base (a purine) attached to a sugar molecule ( ribose ). (selfhacked.com)
- Adenosine is an endogenous purine nucleoside, composed of nucleobase adenine and β-D-ribose moieties. (turkupetcentre.net)
- Adenosine is rapidly catabolized by adenosine deaminases to inosine, which can be further deribosylated by purine nucleoside phosphorylase to hypoxantine . (turkupetcentre.net)
- Methotrexate ( MTX ) increases adenosine release by inhibition of purine metabolism. (turkupetcentre.net)
Pyruvate Kinase1
- Mitapivat is a pyruvate kinase activator that acts by allosterically binding to the pyruvate kinase tetramer and increasing pyruvate kinase activity. (conduent.com)
Adenylate Kinase1
- adenylate kinase 1 [Source:HG. (gsea-msigdb.org)
Dipyridamole1
- Adenosine is antagonized competitively by methylxanthines such as caffeine and theophylline, and potentiated by blockers of nucleoside transport such as dipyridamole. (nih.gov)
Inhibits1
- Adenosine deaminase treatment abolished the suppressive effect of HS, indicating that HS inhibits neutrophils through adenosine generation. (escholarship.org)
Decreases3
- In the brain, excess adenosine decreases alertness and causes sleepiness. (wikipedia.org)
- When larger doses are given by infusion, adenosine decreases blood pressure by decreasing peripheral resistance. (nih.gov)
- As ATP (energy) decreases, adenosine increases and tells the body to start conserving energy. (selfhacked.com)
Endogenous nucleoside2
- Adenosine is an endogenous nucleoside occurring in all cells of the body. (nih.gov)
- Adenosine is an endogenous nucleoside found in every cell of the body. (selfhacked.com)
Transporters1
- typically, reticulum of deaminase by kinase extends the download of primary transporters by docking the brain of RhoA. (evakoch.com)
Glutamate1
- Adenosine modulates the release and uptake of glutamate . (turkupetcentre.net)
Nucleotide1
- Once an adenosine nucleotide is converted to an inosine, it acts in a manner similar to a guanosine nucleotide, with a number of potential consequences [ 4 ]. (biomedcentral.com)
Uric acid1
- Endothelial cells have a very active transport system for adenosine, and also high rate of metabolism, converting adenosine to inosine, hypoxanthine, xanthine, uric acid, and other compounds. (turkupetcentre.net)
Rapidly3
- Intravenously administered adenosine is rapidly cleared from the circulation via cellular uptake, primarily by erythrocytes and vascular endothelial cells. (nih.gov)
- Once inside the cell, adenosine is rapidly phosphorylated by adenosine kinase to adenosine monophosphate, or deaminated by adenosine deaminase to inosine. (globalrph.com)
- Adenosine acts quickly and is rapidly broken down afterward. (selfhacked.com)
Inhibition1
Accumulation3
- Concentration of cAMP in equine neutrophils induced by 5 adenosine A 2A receptor agonists (A) and effects of ZM241385 and MRS1706 on ATL313-induced accumulation of cAMP (B). Each point represents the mean ± SEM of 3 replicates. (avma.org)
- In the absence of functional ADA, there is systemic accumulation of adenosine and deoxyadenosine. (medilib.ir)
- 60 known mutations) results in accumulation of adenosine , which is converted to its ribonucleotide and deoxyribonucleotide (dATP) forms by cellular kinases. (msdmanuals.com)
Inflammation2
- Adenosine (ADO) is an endogenous homeostatic inhibitory neuromodulator that reduces cellular excitability at sites of tissue injury and inflammation. (axonmedchem.com)
- adenosine and adenosine agonists attenuate inflammation , and induce vasodilation and angiogenesis in the cardiovascular system . (turkupetcentre.net)
Enzymes1
- All of these enzymes contain a conserved deaminase domain, shown in blue. (biomedcentral.com)
Hepatic2
- As adenosine requires no hepatic or renal function for its activation or inactivation, hepatic and renal failure would not be expected to alter its effectiveness or tolerability. (nih.gov)
- Adenosine is a potent vasodilator in most vascular beds, except in renal afferent arterioles and hepatic veins where it produces vasoconstriction. (globalrph.com)
Lactate1
- Muscle cramping The cause of cramping is unknown, but may be related to elevated lactate, increased calcium signaling across the sarcoplasmic reticulum caused by membrane instability from reduced levels of ATP, or increased levels of free adenosine. (wikipedia.org)
Isoform1
- Adenosine deaminase isoform 1 (ADA1) is found in most cells, including blood cells. (turkupetcentre.net)
Gene2
- In contrast, Kohn said, "Mother Nature controls the expression of [the BTK ] gene very precisely," referencing the gene, Bruton's tyrosine kinase ( BTK ), that is disrupted in XLA. (primaryimmune.org)
- other specificity is tiny gene activity and distribution browser through the kinase of the R-RasGAP complex ileal to suitable or through the functionality of RhoA. (evakoch.com)
Increases2
- A lack of ADK increases adenosine inside cells and has been associated with diabetes, epilepsy, and cancer. (selfhacked.com)
- Adenosine increases the permeability of the blood-brain barrier . (turkupetcentre.net)
Mechanism1
Deoxyadenosine1
Pathway1
- In this article, we explore adenosine's importance to health, factors that increase adenosine, and how the so-called adenosinergic pathway impacts health. (selfhacked.com)
Bolus4
- Adenosine Injection, USP is a sterile solution for rapid bolus intravenous injection. (nih.gov)
- The intravenous bolus dose of 6 or 12 mg adenosine usually has no systemic hemodynamic effects. (nih.gov)
- A cumulative 60% of patients with paroxysmal supraventricular tachycardia had converted to normal sinus rhythm within one minute after an intravenous bolus dose of 6 mg adenosine (some converted on 3 mg and failures were given 6 mg), and a cumulative 92% converted after a bolus dose of 12 mg. (nih.gov)
- Each I.V. bolus of adenosine should be followed with normal saline flush. (globalrph.com)