KinaseEnzyme glycogen phosphorylaseInhibitorsPurine nucleoside phUridine phosphorylasePYGLGlucagonThymidineGlycogen synthaseSubunitPhosphorylationMetabolismGlucoseHypoglycemiaEnzymesProteinHepatomegalyCatalyzesIsoformsGeneEmbryonicSubunitsBiochemicalRegulationDimerMuscle and liverSkeletal musclesAbundantActivityTissueMusclesFormFoundGSDsChainsFunctionDiseaseSymptoms
Kinase4
- 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)
- 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)
- Pyruvate kinase regulation is important for controlling the flux of metabolites, such as fructose (in liver), which enter glycolysis after the PFK step. (flashcardmachine.com)
- Activates phosphorylase kinase and cyclic nucleotide-independent protein kinase. (lookformedical.com)
Enzyme glycogen phosphorylase2
- This process is accomplished by activating the enzyme glycogen phosphorylase. (tripod.com)
- For example, the original observations made by Fisher and Krebs concerning the regulation of the liver enzyme glycogen phosphorylase were published in Biochimica Biophysica Acta and The Journal of Biological Chemistry , journals that are deemed medium-impact. (universityaffairs.ca)
Inhibitors2
- 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)
Purine nucleoside ph1
- 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 phosphorylase1
PYGL4
- 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)
Glucagon2
- 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)
Thymidine1
- 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 synthase1
- These mice have reduced glycogen stores in adipose tissue, liver, heart, and skeletal muscle, corresponding with decreased glycogen synthase activity and glycogen synthesis rate. (jci.org)
Subunit1
- The allosteric site of AMP binding on muscle isoforms of glycogen phosphorylase are close to the subunit interface just like Ser14. (wikipedia.org)
Phosphorylation3
- 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)
Metabolism1
- 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)
Glucose7
- 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)
Hypoglycemia2
- 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)
Enzymes2
- 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)
Protein1
- The glycogen phosphorylase monomer is a large protein, composed of 842 amino acids with a mass of 97.434 kDa in muscle cells. (wikipedia.org)
Hepatomegaly1
- Liver biopsy may be required to diagnose the cause of hepatomegaly. (medscape.com)
Catalyzes1
- Glycogen phosphorylase (GP) catalyzes the first step of glycogenolysis and is a molecular target for the development of antihyperglycemics. (bvsalud.org)
Isoforms1
- The ACN extract potently inhibits muscle and liver isoforms of GP. (bvsalud.org)
Gene1
- Several mutations of the liver glycogen phosphorylase gene are reported. (medscape.com)
Embryonic1
- 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)
Subunits1
- The enzyme phosphorylates a specific serine residue in each of the subunits of the dimeric phosphorylase b. (expasy.org)
Biochemical2
- Biochemical assay reveals normal phosphorylase activity. (medscape.com)
- In some cases, liver transplantation may abolish biochemical abnormalities. (medscape.com)
Regulation1
- With Steve Mayer, I worked on glycogen phosphorylase and its regulation by catecholamines and cocaine. (vanderbilt.edu)
Dimer1
- The glycogen phosphorylase dimer has many regions of biological significance, including catalytic sites, glycogen binding sites, allosteric sites, and a reversibly phosphorylated serine residue. (wikipedia.org)
Muscle and liver3
- Since 1 gram of carbohydrate contains 4 Calories, the body stores approximately 2000 Calories in the form of muscle and liver glycogen. (tripod.com)
- The glycogen stored in muscle and liver comes from dietary carbohydrates if sufficient quantities of dietary carbohydrates are consumed. (tripod.com)
- These hormones activate both muscle and liver glycogen phosphorylase. (tripod.com)
Skeletal muscles1
- 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)
Abundant1
- 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)
Activity2
- 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)
Tissue1
- 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)
Muscles3
- The average person would store about 400 grams of glycogen in their muscles and 100 grams in their liver. (tripod.com)
- Glycogen phosphorylase is activated automatically when muscles begin to contract. (tripod.com)
- Since glycogen is mainly stored in the muscles, liver or both, GSDs usually affect functioning in these areas, with some GSDs also affecting the heart. (iamgsd.org)
Form2
- Calcium converts the inactive form of glycogen phosphorylase to a more active form of glycogen phosphorylase. (tripod.com)
- For muscle phosphorylase but not liver phosphorylase, this is accompanied by a further dimerization to form a tetrameric phosphorylase. (expasy.org)
Found2
- In mammals, the major isozymes of glycogen phosphorylase are found in muscle, liver, and brain. (wikipedia.org)
- The liver phosphorylase enzyme is found in the liver and in red blood cells. (medscape.com)
GSDs2
- 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)
Chains1
- Glycogen phosphorylase can act only on linear chains of glycogen (α1-4 glycosidic linkage). (wikipedia.org)
Function2
- Liver function studies are indicated and may reveal evidence of hepatic injury. (medscape.com)
- Liver function studies are indicated. (medscape.com)
Disease3
- 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)
Symptoms1
- 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)