GlycogenInhibitorsKinaseEnzymeDeficiencyUridinePolyclonal antibodyNucleosideAdenosineCatalyticBiochemistryPhosphateGliostatinLiverReactionRabbitMuscleExoribonucleasePotentGeneActivityHumanModificationBreast CancerPurine nucleosideThymidineKinasePhosphorolysisNucleoside phosphorylasesAdenosineInhibitorCatalysisMiceEscherichiaTumorsGeneticTargetHttpsActiveAnalysisModel
Glycogen13
- Phosphorylase is also a common name used for glycogen phosphorylase in honor of Earl W. Sutherland Jr., who in the late 1930s discovered it as the first phosphorylase. (wikipedia.org)
- Phosphorylase a is the more active R form of glycogen phosphorylase that is derived from the phosphorylation of the less active R form, phosphorylase b with associated AMP. (wikipedia.org)
- Some disorders are related to phosphorylases: Glycogen storage disease type V - muscle glycogen Glycogen storage disease type VI - liver glycogen Hydrolase Nelson DL, Lehninger AL, Cox MM (2005). (wikipedia.org)
- The design of glycogen phosphorylase (GP) inhibitors targeting the catalytic site of the enzyme is a promising strategy for a better control of hyperglycaemia in the context of type 2 diabetes. (rsc.org)
- Here, we used a novel glycogen phosphorylase inhibitor, CP-316,819 ([ R - R *, S *]-5-chloro- N -[2-hydroxy-3-(methoxymethylamino)-3-oxo-1-(phenylmethyl)propyl]-1 H -indole-2-carboxamide), that causes glycogen accumulation under normoglycemic conditions but permits glycogen utilization when glucose concentrations are low. (aspetjournals.org)
- The enzyme couples muscle contraction with energy production via glycogenolysis--glycolysis by catalyzing the Ca(2+)-dependent phosphorylation and activation of glycogen phosphorylase b. (expasy.org)
- Magnetic Luminex Assay Kit for Glycogen Phosphorylase, Liver (PYGL) ,etc. (uscnk.com)
- Phosphorylases are enzymes that catalyze the addition of a phosphate group from an inorganic phosphate (phosphate+hydrogen) to an acceptor.They include allosteric enzymes that catalyze the production of glucose-1-phosphate from a glucan such as glycogen, starch or maltodextrin. (targetmol.com)
- CP-91149 is a selective glycogen phosphorylase (GP) inhibitor with IC50 of 0.13 μM in the presence of glucose, 5- to 10-fold less potent in the absence of glucos. (targetmol.com)
- Ingliforib is a novel glycogen phosphorylase inhibitor,has antihyperglycemic and cardioprotective properties. (targetmol.com)
- Glycogen phosphorylase kinase β-subunit (PHKB) is a regulatory subunit of phosphorylase kinase (PHK), involving in the activation of glycogen phosphorylase (GP) and the regulation of glycogen breakdown. (ijbs.com)
- As is the case with rabbit muscle phosphorylase a the dimer a is the active form of frog muscle phosphorylase a with glycogen as the substrate. (northwestern.edu)
- The extract showed good inhibition toward rat intestinal maltase and rabbit glycogen phosphorylase (GP) b, with IC 50 values of 45 and 7.4 μg/ml, respectively. (breakawaymatcha.com)
Inhibitors1
- Application of crystallographic and modeling methods in the design of purine nucleoside phosphorylase inhibitors. (lu.se)
Kinase8
- In more general terms, phosphorylases are enzymes that catalyze the addition of a phosphate group from an inorganic phosphate (phosphate + hydrogen) to an acceptor, not to be confused with a phosphatase (a hydrolase) or a kinase (a phosphotransferase). (wikipedia.org)
- Phosphorylase kinase is a polymer of 16 subunits, four each of alpha, beta, gamma and delta. (nih.gov)
- A novel PHKA1 mutation associating myopathy and cognitive impairment: Expanding the spectrum of phosphorylase kinase b (PhK) deficiency. (nih.gov)
- Sodium Monofluorophosphate is a competitive inhibitor of pyruvate kinase and alkaline phosphatase, which also irreversibly inhibits phosphorylase phosphatase. (targetmol.com)
- Phosphorylase kinase is a 1.3 MDa hexadecameric holoenzyme. (phosphorylase-signal.com)
- Yang W, Zhang C, Li Y, Jin A, Sun Y, Yang X, Wang B, Guo W. Phosphorylase Kinase β Represents a Novel Prognostic Biomarker and Inhibits Malignant Phenotypes of Liver Cancer Cell. (ijbs.com)
- Frog muscle phosphorylase b can be converted into the phosphorylated form (a) by rabbit skeletal muscle phosphorylase b kinase, adenosine triphosphate (ATP), and Mg 2+ . (northwestern.edu)
- Frog muscle phosphorylase b can be converted into the phosphorylated form (a) by rabbit skeletal muscle phosphorylase b kinase, adenosine triphosphate (ATP), and Mg2+. (northwestern.edu)
Enzyme7
- Uridine phosphorylase (UP) is a key enzyme in the pyrimidine salvage pathway that catalyses the reversible phosphorolysis of uridine to uracil and ribose 1-phosphate. (rcsb.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)
- The enzyme phosphorylates a specific serine residue in each of the subunits of the dimeric phosphorylase b. (expasy.org)
- The enzyme differs from mammalian muscle phosphorylase a in that the equilibrium between phosphorylase a dimer and phosphorylase a tetramer is shifted in the direction of the dimer. (northwestern.edu)
- Purine nucleoside phosphorylase (PNP) is the key enzyme of the purine salvage pathway. (biospot.eu)
- The TYMP gene provides instructions for making an enzyme called thymidine phosphorylase. (medlineplus.gov)
- First, a sucrose phosphorylase (SPase) mutant with higher enzyme activity was obtained by experimental screening . (bvsalud.org)
Deficiency10
- Purine nueoside phosphorylase (PNP) deficiency causes a form of severe combined immunodeficiency (SCID) characterized by profound T cell deficiency, failure to thrive (FTT), recurrent deep seeded infection, developmental delay, progressive neurological deterioration, and autoimmune complications. (medscape.com)
- [ 2 , 3 ] One disorder is adenosine deaminase (ADA) deficiency, which is Online Mendelian Inheritance in Man (OMIM) subject number 102700, and the other is purine nucleoside phosphorylase (PNP) deficiency, which is OMIM subject number 164050. (medscape.com)
- In purine nucleoside phosphorylase deficiency, similar changes occur, resulting in elevated deoxy-GTP (dGTP) levels. (medscape.com)
- In a further description of the mechanism of T-cell depletion in purine nucleoside phosphorylase deficiency, Arpaia et al reported increased in vivo apoptosis of T cells and increased in vitro sensitivity to gamma irradiation in a murine model. (medscape.com)
- [ 4 ] The immune deficiency in purine nucleoside phosphorylase deficiency may be the result of inhibited mitochondrial DNA repair due to the accumulation of dGTP in the mitochondria. (medscape.com)
- Purine nucleoside phosphorylase (PNP) deficiency, a rare autosomal recessive metabolic disease causes combined immunodeficiency and developmental delay, hypotonia, and spasticity. (frontiersin.org)
- Purine nucleoside phosphorylase (PNP) deficiency (OMIM 613179) is a rare autosomal recessive metabolic disease leading to combined immunodeficiency and neurological abnormalities, which may include developmental delay, hypotonia, and spasticity ( 1 , 2 ). (frontiersin.org)
- INTRODUCTION - Purine nucleoside phosphorylase (PNP) deficiency (MIM #613179) is a rare, autosomal recessive, inborn error of immunity (IEI) [ 1-3 ]. (medilib.ir)
- Diagnosis of purine nucleoside phosphorylase deficiency is by DNA analysis. (msdmanuals.com)
- Treatment of purine nucleoside phosphorylase deficiency is with bone marrow or stem cell transplantation. (msdmanuals.com)
Uridine1
- Crystal structures of escherichia coli uridine phosphorylase in two native and three complexed forms reveal basis of substrate specificity, induced conformational changes and influence of potassium. (expasy.org)
Polyclonal antibody2
- Thymidine Phosphorylase antibody LS-C699697 is a Cy3-conjugated rabbit polyclonal antibody to rat Thymidine Phosphorylase (TP) (aa33-290). (lsbio.com)
- The following product was used in this experiment: Thymidine Phosphorylase Polyclonal Antibody from Thermo Fisher Scientific, catalog # PA5-17098, RRID AB_10984207. (thermofisher.cn)
Nucleoside2
- In both adenosine deaminase and purine nucleoside phosphorylase deficiencies, thymocytes are thought to be selectively destroyed because of elevated levels of dATP and dGTP. (medscape.com)
- These hexameric structures confirm the overall structural similarity of UP to E.coli purine nucleoside phosphorylase (PNP) whereby, in the presence of substrate, each displays a closed conformation resulting from a concerted movement that closes the active site cleft. (rcsb.org)
Adenosine1
- Previous studies have revealed that the adenosine 5′-monophosphate phosphorylase of Thermococcus kodakarensis ( Tk AMPpase) forms large soluble multimers with high thermal stability. (tu-berlin.de)
Catalytic1
- Modification of regulatory and catalytic properties of phosphorylase-b by irradiation and heat. (nebraska.edu)
Biochemistry1
- In biochemistry, phosphorylases are enzymes that catalyze the addition of a phosphate group from an inorganic phosphate (phosphate+hydrogen) to an acceptor. (wikipedia.org)
Phosphate4
- Phosphorylases should not be confused with phosphatases, which remove phosphate groups. (wikipedia.org)
- Other names in common use include UAR phosphorylase , and urate-ribonucleotide:phosphate D-ribosyltransferase . (wikipedia.org)
- Unexpectedly, the reaction is stimulated by inorganic phosphate, and our data reveal that MbcT is a NAD+ phosphorylase. (figshare.com)
- There are 4 moles of pyridoxal 5′-phosphate and 4 moles of orthophosphate bound to each 370,000 g of frog skeletal muscle phosphorylase a. (northwestern.edu)
Gliostatin1
- A platelet-derived endothelial growth factor (PD-ECGF), same as thymidine phosphorylase (TP) or gliostatin. (thermofisher.cn)
Liver1
- For muscle phosphorylase but not liver phosphorylase, this is accompanied by a further dimerization to form a tetrameric phosphorylase. (expasy.org)
Reaction1
- Recombinant human thymidine phosphorylase catalyzes the reaction of arsenate with thymidine to form thymine and 2-deoxyribose 1-arsenate, which rapidly decomposes to 2-deoxyribose and inorganic arsenate. (elsevierpure.com)
Rabbit2
- A redetermination of the molecular weight of rabbit muscle phosphorylase b by sedimentation equilibrium and by sedimentation velocity and diffusion gave a value of 185,000. (northwestern.edu)
- The amino acid compositions of frog, rabbit, and human skeletal muscle phosphorylases are quite similar, but frog and rabbit muscle enzymes differ immunologically. (northwestern.edu)
Muscle4
- The purification of frog skeletal muscle phosphorylase is described. (northwestern.edu)
- Both types (b and a) of frog muscle phosphorylase appear to be essentially homogenous by a variety of criteria. (northwestern.edu)
- The molecular weight of frog muscle phosphorylase b was determined by sedimentation equilibrium and found to be 188,000 g. (northwestern.edu)
- Several kinetic parameters of frog muscle phosphorylase a have been determined. (northwestern.edu)
Exoribonuclease1
- Polynucleotide phosphorylase (PNPase) is a processive exoribonuclease that contributes to messenger RNA turnover and quality control of ribosomal RNA precursors in many bacterial species. (port.ac.uk)
Potent1
- The expression of thymidine phosphorylase (TP), a potent chemotactic factor for endothelial cells, was studied in 60 adenocarcinomas of the gallbladder, by use of immunohistochemical techniques. (ox.ac.uk)
Gene1
- TYMP gene variants greatly reduce or eliminate the activity of thymidine phosphorylase. (medlineplus.gov)
Activity1
- A loss of thymidine phosphorylase activity and the resulting buildup of thymidine disrupt the usual maintenance and repair of mtDNA. (medlineplus.gov)
Human2
- The structure of the transition state confirms that human thymidine phosphorylase proceeds through an S N 2-like transition state with bond orders of 0.50 to the thymine leaving group and 0.33 to the attacking oxygen nucleophile. (elsevierpure.com)
- Birck, MR & Schramm, VL 2004, ' Nucleophilic Participation in the Transition State for Human Thymidine Phosphorylase ', Journal of the American Chemical Society , vol. 126, no. 8, pp. 2447-2453. (elsevierpure.com)
Modification1
- Arbutin Production through the Engineering of Sucrose Phosphorylase Combined with Engineered Cell Modification. (bvsalud.org)
Breast Cancer1
- Contrary findings exist according to the prognostic and predictive impact of thymidine phosphorylase (TP) expression in breast cancer. (uni-luebeck.de)
Purine nucleoside14
- When Do Symptoms of Purine nucleoside phosphorylase deficiency Begin? (nih.gov)
- Purine nucleoside phosphorylase deficiency is a disorder of the immune system called an immunodeficiency. (medlineplus.gov)
- The shortage of immune system cells in people with purine nucleoside phosphorylase deficiency results in repeated and persistent infections typically beginning in infancy or early childhood. (medlineplus.gov)
- The infections can be very serious or life-threatening, and without successful treatment to restore immune function, children with purine nucleoside phosphorylase deficiency usually do not survive past childhood. (medlineplus.gov)
- Infants with purine nucleoside phosphorylase deficiency typically grow more slowly than healthy babies. (medlineplus.gov)
- People with purine nucleoside phosphorylase deficiency are also at increased risk of developing autoimmune disorders, which occur when the immune system malfunctions and attacks the body's tissues and organs. (medlineplus.gov)
- Purine nucleoside phosphorylase deficiency is caused by mutations in the PNP gene. (medlineplus.gov)
- The shortage of T cells and sometimes B cells results in the immune problems characteristic of purine nucleoside phosphorylase deficiency. (medlineplus.gov)
- Damage to brain cells caused by buildup of dGTP is thought to underlie the neurological problems that occur in some people with purine nucleoside phosphorylase deficiency. (medlineplus.gov)
- Development and validation of a 2nd tier test for identification of purine nucleoside phosphorylase deficiency patients during expanded newborn screening by liquid chromatography-tandem mass spectrometry. (medscape.com)
- Immunodeficiency diseases caused by adenosine deaminase deficiency and purine nucleoside phosphorylase deficiency. (medscape.com)
- Available at https://ghr.nlm.nih.gov/condition/purine-nucleoside-phosphorylase-deficiency . (medscape.com)
- Diagnosis of purine nucleoside phosphorylase deficiency is by DNA analysis. (msdmanuals.com)
- Treatment of purine nucleoside phosphorylase deficiency is with bone marrow or stem cell transplantation. (msdmanuals.com)
Thymidine8
- Patients with high thymidine phosphorylase ( TP) gene expression (n = 62, dotted line) in tumour tissues exhibited significantly longer time to progression compared with patients with low expression (n = 63, solid line). (medscape.com)
- TP Thymidine Phosphorylase. (medscape.com)
- Patient and tumour characteristics subgrouped according to high or low thymidine phosphorylase expression. (medscape.com)
- Prognoses in cases of uterine cervical squamous cell carcinoma treated with radiotherapy were investigated in association with immunohistochemical expression of an angiogenic factor, thymidine phosphorylase (TP). (bmj.com)
- The angiogenic factor thymidine phosphorylase (TP) is highly expressed in human monocytes and macrophages, and its expression has been linked to the pathology and progression of solid tumors, rheumatoid arthritis, and gastric ulcers. (elsevierpure.com)
- Zhu, GH & Schwartz, EL 2003, ' Expression of the Angiogenic Factor Thymidine Phosphorylase in THP-1 Monocytes: Induction by Autocrine Tumor Necrosis Factor-α and Inhibition by Aspirin ', Molecular Pharmacology , vol. 64, no. 5, pp. 1251-1258. (elsevierpure.com)
- Thymidine phosphorylase (TP) anabolises formation of pyrimidine nucleosides available for DNA synthesis, whereas dihydropyrimidine dehydrogenase (DPD) catabolises the degradation of pyrimidine bases, thereby reducing levels of uracil and thymine available for DNA synthesis. (elsevierpure.com)
- Focal expression of thymidine phosphorylase associates with CD31 positive lymphocytic aggregation and local neo-angiogenesis in non-small cell lung cancer. (ox.ac.uk)
Kinase4
- In more general terms, phosphorylases are enzymes that catalyze the addition of a phosphate group from an inorganic phosphate (phosphate + hydrogen) to an acceptor, not to be confused with a phosphatase (a hydrolase) or a kinase (a phosphotransferase). (wikipedia.org)
- Phosphorylase Kinase (PhK) is an essential regulatory enzyme in the glycogenolysis cascade. (ku.edu)
- Phosphorylase kinase (PhK) deficiency causing glycogen storage disease type IX (GSD IX) results from deficiency of the enzyme phosphorylase b kinase, which has a major regulatory role in the breakdown of glycogen. (nih.gov)
- Phosphorylase kinase is a 1.3 MDa hexadecameric holoenzyme. (phosphorylase-signal.com)
Phosphorolysis1
- A class of CAZymes, known as glycoside phosphorylases (GPs) catalyze the reversible phosphorolysis of glycosidic bonds, releasing sugar 1-phosphates and have considerable potential as catalysts for the assembly of useful carbohydrates for products ranging from functional foods and prebiotics to novel materials. (ubc.ca)
Nucleoside phosphorylases1
- Biocatalytic nucleoside (trans-)glycosylations catalyzed by nucleoside phosphorylases have graduated to a practical and convenient approach to the preparation of modified nucleosides, which are important pharmaceuticals for the treatment of various cancers and viral infections. (chemrxiv.org)
Adenosine2
- Adenosine deaminase and purine nucleoside phosphorylase deficiencies: evaluation of therapeutic interventions in eight patients. (medscape.com)
- Previous studies have revealed that the adenosine 5′-monophosphate phosphorylase of Thermococcus kodakarensis ( Tk AMPpase) forms large soluble multimers with high thermal stability. (tu-berlin.de)
Inhibitor2
- A mixed type inhibitor of muscle glycogen phosphorylase (K i = 930 nM). (emdmillipore.com)
- The fourth-generation immucillin derivative (DI4G), a potent inhibitor of purine nucleoside phosphorylase, has been proposed as a promising agent in the treatment of diseases associated with T cell activation. (fiocruz.br)
Catalysis1
- Catalysis of the reaction: 4 ATP + 2 phosphorylase b = 4 ADP + phosphorylase a. (yeastrc.org)
Mice1
- Evidence from purine nucleoside phosphorylase-deficient mice. (medscape.com)
Escherichia1
- Designer gene therapy using an Escherichia coli purine nucleoside phosphorylase/prodrug system. (expasy.org)
Tumors1
- It is rare for this deletion to occur in primary prostate tumors, yet approximately 10% express decreased levels of methylthioadenosine phosphorylase (MTAP) mRNA. (nih.gov)
Genetic1
- Genetic variation in genes associated with arsenic metabolism: glutathione S-transferase omega 1-1 and purine nucleoside phosphorylase polymorphisms in European and indigenous Americans. (nih.gov)
Target1
- Glycogen phosphorylase (GP) is a promising molecular target for the treatment of Type 2 diabetes. (eurekaselect.com)
Https1
- Available at https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=EN&Expert=760 . (medscape.com)
Active1
- Phosphorylase a is the more active R form of glycogen phosphorylase that is derived from the phosphorylation of the less active R form, phosphorylase b with associated AMP. (wikipedia.org)
Analysis1
- Our multifaceted analysis further describes the kinetic implications of this in situ reactant esterification for a model phosphorylase. (chemrxiv.org)
Model1
- The glucose-induced switch between glycogen phosphorylase and glycogen synthase in the liver is investigated by means of a theoretical approach based on a minimal, bicyclic cascade model involving the reversible phosphorylation of the two enzymes. (cellml.org)