• Application of crystallographic and modeling methods in the design of purine nucleoside phosphorylase inhibitors. (lu.se)
  • Structure-based design and synthesis of two biphenyl-N-acyl-ß-d-glucopyranosylamine derivatives as well as their assessment as inhibitors of human liver glycogen phosphorylase (hlGPa, a pharmaceutical target for type 2 diabetes) is presented. (bvsalud.org)
  • 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)
  • 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)
  • Magnetic Luminex Assay Kit for Glycogen Phosphorylase, Liver (PYGL) ,etc. (uscnk.com)
  • GSD VI is caused by deficient activity of hepatic glycogen phosphorylase, an enzyme encoded by the PYGL gene, which is located on chromosome 14q21-q22. (medscape.com)
  • The phosphorylated PYGL dimer (a form) of glycogen phosphorylase catalyzes the reaction of orthophosphate and glycogen-glycogenin 1 to form D-glucose 1-phosphate and limit dextrin-glycogenin 1. (reactome.org)
  • In the body, this reaction takes place in the liver where its dependence on hormonally stimulated PYGL phosphorylation (and lack of sensitivity to AMP) allow glucose mobilization in response to a demand for glucose from the rest of the body (Newgard et al. (reactome.org)
  • The Glucagon-Sensitive Adenyl Cyclase System in Plasma Membranes of Rat Liver: II. (nih.gov)
  • Earl Sutherland's work at the time made it a natural transition for me to work with him, as he had worked out the mechanism by which epinephrine and glucagon activate phosphorylase in the liver, including the discovery of cyclic AMP. (vanderbilt.edu)
  • Capecitabine is converted in the liver to 5-fluoro-5'- deoxyuridine (55FdU) and this is converted to 5-FU by thymidine phosphorylase whose over expression is induced by radiotherapy. (dcu.ie)
  • Glycogen phosphorylase is also studied as a model protein regulated by both reversible phosphorylation and allosteric effects. (wikipedia.org)
  • The structural change associated with phosphorylation, and with the conversion of phosphorylase b to phosphorylase a, is the arrangement of the originally disordered residues 10 to 22 into α helices. (wikipedia.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)
  • Mississippi Metabolics Foundation (MMF) was founded to raise awareness, educate, and provide support to those living or caring for someone with genetic metabolic disorders/inborn errors of metabolism (IEM). (globalgenes.org)
  • Glycogen phosphorylase catalyzes the rate-limiting step in glycogenolysis in animals by releasing glucose-1-phosphate from the terminal alpha-1,4-glycosidic bond. (wikipedia.org)
  • Glycogen phosphorylase breaks up glycogen into glucose subunits (see also figure below): (α-1,4 glycogen chain)n + Pi ⇌ (α-1,4 glycogen chain)n-1 + α-D-glucose-1-phosphate. (wikipedia.org)
  • Some sugars like fructose (the primary sugar in fruits) when consumed in significant amounts (the amount varies but for fructose it is typically 50g or more per meal) may enter the bloodstream in their native form and must be converted to glucose by the liver. (tripod.com)
  • When muscle and liver glycogen levels are replenished, the excess glucose is burned preferentially to fat. (tripod.com)
  • Glycogen phosphorylase removes glucose units, one at a time, from the glycogen chain. (tripod.com)
  • Excess glucose in the body is stored as glycogen in the liver and serves as a reserve for glucose needs, especially during fasting state. (medscape.com)
  • Excess glucose is stored as glycogen in our skeletal muscles and our liver. (iamgsd.org)
  • Hepatic phosphorylase, which is found in the liver and red blood cells, is deficient in GSD VI, which results in glycogen accumulation in the liver and subsequent hypoglycemia. (medscape.com)
  • In patients with Hers disease, defective liver phosphorylase results in hepatomegaly and hypoglycemia. (medscape.com)
  • Glycogen phosphorylase is one of the phosphorylase enzymes (EC 2.4.1.1). (wikipedia.org)
  • One of the most common resistance mechanisms against aminoglycosides is the production of aminoglycoside-modifying enzymes, such as aminoglycoside acetyltransferases, aminoglycoside phosphorylases, and aminoglycoside adenyltransferases ( 5 ), which are mainly mediated by transferable large plasmids. (cdc.gov)
  • Liver biopsy may be required to diagnose the cause of hepatomegaly. (medscape.com)
  • Glycogen phosphorylase (GP) catalyzes the first step of glycogenolysis and is a molecular target for the development of antihyperglycemics. (bvsalud.org)
  • The ACN extract potently inhibits muscle and liver isoforms of GP. (bvsalud.org)
  • The brain type is predominant in adult brain and embryonic tissues, whereas the liver and muscle types are predominant in adult liver and skeletal muscle, respectively. (wikipedia.org)
  • Because carbohydrates play such an important role in producing energy for the body, they are stored in the form of glycogen in both skeletal muscles and in the liver. (tripod.com)
  • Although the liver has a higher concentration of glycogen than muscle there is more glycogen stored in muscle tissue because muscle tissue is more abundant than liver tissue. (tripod.com)
  • This change increases phosphorylase activity up to 25% even in the absence of AMP, and enhances AMP activation further. (wikipedia.org)
  • Inhibiting liver UP in humans raises blood uridine levels and produces a protective effect ("uridine rescue") against the toxicity of the chemotherapeutic agent 5-fluorouracil without reducing its antitumour activity. (rcsb.org)
  • an enzyme defective within a particular organ or tissue, such as the liver , muscle , or brain , might lead to low energy production or prevent transport to the part of the body requiring energy. (newworldencyclopedia.org)
  • Liver GSDs may result in an enlarged liver and low blood sugar. (iamgsd.org)
  • At present there are around 20 distinct forms of GSD, split approximately half and half between liver and muscle GSDs. (iamgsd.org)
  • Glycogen phosphorylase can act only on linear chains of glycogen (α1-4 glycosidic linkage). (wikipedia.org)
  • Liver function studies are indicated and may reveal evidence of hepatic injury. (medscape.com)
  • Liver function studies are indicated. (medscape.com)
  • To improve the lives of individuals and families impacted by liver disease through promoting innovation, encouraging collaboration, and scaling optimal approaches to help eradicate liver diseases. (globalgenes.org)
  • The Neuromuscular Disease Foundation's (NDF) mission is to enhance the quality of the lives of people living with GNE Myopathy (also known as HIBM) through advocacy, education, outreach, and funding clinical research focused on treatments and a cure. (globalgenes.org)
  • There are over a dozen forms of glycogen storage disease (GSD), and a number of sub-forms, that can affect skeletal muscle, the liver, or the heart. (iamgsd.org)
  • Given the number of metabolic disorders and the range of systems affected, these disorders are manifested in a wide array of symptoms of varying severity, ranging from recurrent vomiting, lethargy, and muscle weakness, to liver and heart failure, developmental delay, and mental retardation. (newworldencyclopedia.org)