Glycogen
Wikimedia Commons has media related to Glycogen. "Glycogen storage disease". McArdle's Diseases. Glycogen at the U.S. National ... In humans, glycogen is made and stored primarily in the cells of the liver and skeletal muscle. In the liver, glycogen can make ... Glycogen functions as one of two forms of energy reserves, glycogen being for short-term and the other form being triglyceride ... Glycogen is cleaved from the nonreducing ends of the chain by the enzyme glycogen phosphorylase to produce monomers of glucose- ...
Glycogen phosphorylase
... breaks up glycogen into glucose subunits (see also figure below): (α-1,4 glycogen chain)n + Pi ⇌ (α-1,4 ... Glycogen phosphorylase kinase activates glycogen phosphorylase in the same manner mentioned previously. Glycogen phosphorylase ... Evidence that dephosphorylation of glycogen phosphorylase and glycogen synthase in the glycogen and microsomal fractions of rat ... Glycogen phosphorylase can act only on linear chains of glycogen (α1-4 glycosidic linkage). Its work will immediately come to a ...
Glycogen body
... but it does not seem to be related to the normal function of glycogen in animals, which is the storage of energy. Glycogen ... A glycogen body is an oval structure in the spinal cord of birds that is made of specialized cells that contain large amounts ... Imagawa, T; Shogaki, K; Uehara, M (October 2006). "Interaction between glycogen body cell and neuron: examination in co-culture ... of glycogen. Housed within the synsacrum, the function of this structure is not known, ...
Glycogen synthase
The control of glycogen synthase is a key step in regulating glycogen metabolism and glucose storage. Glycogen synthase is ... Liver glycogen serves as a storage pool to maintain the blood glucose level during fasting, whereas muscle glycogen synthesis ... This suggests that glycogen synthase plays an important biological role in regulating glycogen/glucose levels and is activated ... However, muscle-specific glycogen synthase activation may lead to excessive accumulation of glycogen, leading to damage in the ...
Glycogen storage disease
Glycogen-Storage Disease Type I Author: Karl S Roth. Updated: Aug 31, 2009 "Glycogen Storage Disease Type I". Association for ... GLYCOGEN STORAGE DISEASE IXa1; GSD9A1 OMIM - Online Mendelian Inheritance in Man "Definition: glycogen storage disease type ... GSD type 0: Although glycogen synthase deficiency does not result in storage of extra glycogen in the liver, it is often ... See inborn errors of carbohydrate metabolism for a full list of inherited diseases that affect glycogen synthesis, glycogen ...
Glycogen debranching enzyme
When glycogen breakdown is compromised by mutations in the glycogen debranching enzyme, metabolic diseases such as Glycogen ... GeneReviews/NCBI/NIH/UW entry on Glycogen Storage Disease Type III OMIM entries on Glycogen Storage Disease Type III Glycogen+ ... In GSD III glycogen breakdown is incomplete and there is accumulation of abnormal glycogen with short outer branches. Most ... Glycogen debranching enzymes assist phosphorylase, the primary enzyme involved in glycogen breakdown, in the mobilization of ...
Glycogen branching enzyme
... the extended glycogen polymer is branched by glycogen branching enzyme to provide glycogen breakdown enzymes, such as glycogen ... Glycogen branching enzyme is an enzyme that adds branches to the growing glycogen molecule during the synthesis of glycogen, a ... Importantly, glycogen synthase can only catalyze the synthesis of α-1,4-glycosidic linkages. Since glycogen is a readily ... highly branched glycogen molecule. Mutations in this gene are associated with glycogen storage disease type IV (also known as ...
glycogen-synthase-D) phosphatase
... glycogen synthetase phosphatase, glycogen synthase phosphatase, glycogen synthase D phosphatase, Mg2+ dependent glycogen ... UDP-glycogen glucosyltransferase phosphatase, UDPglucose-glycogen glucosyltransferase phosphatase, glycogen glucosyltransferase ... The enzyme [glycogen-synthase-D] phosphatase ({EC 3.1.3.42) catalyzes the reaction [glycogen-synthase D] + H2O ⇌ {\displaystyle ... The systematic name is [UDP-glucose:glycogen 4-α-D-glucosyltransferase-D] phosphohydrolase. Other names in common use include ...
Glycogen-branching enzyme deficiency
The human form of the disease is known as glycogen storage disease type IV. Glycogen is a molecular polymer of glucose used to ... Glycogen-branching enzyme deficiency (GBED) is an inheritable glycogen storage disease affecting American Quarter Horses and ... This bond may be broken by amylase when the body wishes to break down glycogen into glucose for energy. Glycogen branching ... This causes low levels of muscle glycogen that is very resistant to amylase. Lacking proper glycogen storage, the horse's brain ...
Glycogen phosphorylase isoenzyme BB
... (abbreviation: GPBB) is an isoenzyme of glycogen phosphorylase. This isoform of the enzyme ... 2005). "Glycogen phosphorylase BB in acute coronary syndromes". Clin. Chem. Lab. Med. 43 (12): 1351-8. doi:10.1515/CCLM. ... Other enzymes related to glycogen phosphorylase are abbreviated as GPLL (liver) and GPMM (muscle). Apple FS, Wu AH, Mair J, et ...
Glycogen storage disease type I
GeneReview/NIH/UW entry on Glycogen Storage Disease Type I Media related to Glycogen storage disease type I at Wikimedia ... excess glycogen storage in the liver and (in some cases) in the kidneys. Because of the glycogen buildup, GSD I patients ... Glycogen Storage Disease Ic - 232240 PARKER, PAUL (1981). "Regression of hepatic adenomas in type Ia glycogen storage disease ... and to add the G6P molecules to the ends of chains of glycogen (glycogen synthesis). Excess G6P is also shunted into production ...
Glycogen storage disease type III
"glycogen storage disease type III". Genetics Home Reference. Retrieved 2016-08-07. "Glycogen storage disease type 3 , Genetic ... Without glycogen debranching enzymes to further convert these branched glycogen polymers to glucose, limit dextrinosis ... Scholia has a topic profile for Glycogen storage disease type III. Media related to Glycogen storage disease type III at ... The gene is responsible for creating glycogen debranching enzyme, which in turn helps in glycogen decomposition. In terms of ...
Glycogen synthase kinase-3 beta
... , (GSK-3 beta), is an enzyme that in humans is encoded by the GSK3B gene. In mice, the enzyme is ... "Entrez Gene: GSK3B glycogen synthase kinase 3 beta". Iwahashi K, Nishizawa D, Narita S, Numajiri M, Murayama O, Yoshihara E, et ... Glycogen synthase kinase-3 (GSK-3) is a proline-directed serine-threonine kinase that was initially identified as a ... Hoeflich KP, Luo J, Rubie EA, Tsao MS, Jin O, Woodgett JR (July 2000). "Requirement for glycogen synthase kinase-3beta in cell ...
Glycogen storage disease type VI
... (GSD VI) is a type of glycogen storage disease caused by a deficiency in liver glycogen ... "Glycogen storage disease type VI". GeneReview/NIH/UW entry on Glycogen Storage Disease Type VI Media related to Glycogen ... Glycogen storage disease due to liver glycogen phosphorylase deficiency". www.orpha.net. Retrieved 11 April 2019. Glycogen- ... Because glycogen is only broken down when stored energy needs to be used, eating frequent meals can prevent the need to break ...
Glycogen storage disease type V
Glycogen storage disease Hitting the wall (muscle fatigue due to glycogen depletion) Inborn errors of carbohydrate metabolism ... Another glycogen storage disease that affects muscle (muscle GSD); Metabolic myopathy other than glycogen storage disease; ... Glycogen storage disease type V (GSD5, GSD-V), also known as McArdle's disease, is a metabolic disorder, one of the metabolic ... "Reaction participants of glycogen phosphorylase". www.rhea-db.org. Retrieved 2020-12-26. NCBI Gene ID 5837: PYGM phosphorylase ...
Glycogen storage disease type 0
Glycogen storage disease due to liver glycogen synthase deficiency Glycogen storage disease due to muscle and heart glycogen ... "Glycogen-Storage Disease Type 0" "Orphanet: Glycogen storage disease due to hepatic glycogen synthase deficiency". www.orpha. ... Glycogen storage disease type 0 is a disease characterized by a deficiency in the glycogen synthase enzyme (GSY). Although ... Glycogen synthetase catalyzes the rate-limiting reaction for glycogen synthesis in the liver by transferring glucose units from ...
Glycogen storage disease type IV
... (GSD IV), or Andersen's Disease, is a form of glycogen storage disease, which is caused by an ... Therefore, glycogen is not made properly and abnormal glycogen molecules accumulate in cells; most severely in cardiac and ... It is a result of the absence of the glycogen branching enzyme, which is critical in the production of glycogen. This leads to ... "Deficiency of glycogen branching enzyme (GBE) activity causes glycogen storage disease type IV (GSD IV), an autosomal recessive ...
Glycogen storage disease type II
"Type II Glycogen Storage Disease". The Association for Glycogen Storage Disease. Archived from the original on June 23, 2012. ... Association of Glycogen Storage Disease in the United States AGSD-UK - Association of Glycogen Storage Disease in the UK AMDA ... of cellular glycogen. The deficiency of this enzyme results in the accumulation of structurally normal glycogen in lysosomes ... Genetics of Glycogen-Storage Disease Type II (Pompe Disease) at eMedicine Van der Ploeg AT, Kroos MA, Willemsen R, Brons NH, ...
Glycogen storage disease type IX
... is a hereditary deficiency of glycogen phosphorylase kinase B that affects the liver and ... glycogen storage disease IXa1 and glycogen storage disease IXa2 that affect the liver of an individual. Mutations in PHKA2 have ... Scholia has a topic profile for Glycogen storage disease type IX. Media related to Glycogen storage disease type IX at ... Glycogen storage disease type IX can be inherited via: X-linked recessive inheritance due to mutations at either PHKA1 or the ...
List of unsolved problems in biology
Glycogen body. The function of this structure in the spinal cord of birds is not known. Arthropod head problem. A long-standing ...
Glossary of diabetes
Glycogenesis The process by which glycogen is formed from glucose. Controlled by insulin. See also: Glycogen. Glycosuria Having ... Glycogen A substance made from multiple glucose molecules. Sometimes called 'animal starch'. It is stored in liver and muscle ... The glucose in liver glycogen is put back into the blood when required. That in muscle cells is not, as they lack the necessary ... starch and glycogen-both chains of glucose molecules).are eventually broken down to glucose during digestion. They eventually ...
Phosphorylase kinase
... triggering a conformational shift which favors the more active glycogen phosphorylase "a" form over the less active glycogen ... 2. Glycogen metabolism". Eur J Biochem. 132 (2): 263-274. doi:10.1111/j.1432-1033.1983.tb07358.x. PMID 6301825. Ingebritsen TS ... Defects in phosphorylase kinase genes are the cause of glycogen storage disease type IX (GSD type IX) and GSD type VI (formerly ... The substrate of PhK, glycogen phosphorylase, had been isolated by Carl and Gerty Cori in the 1930s, who determined that there ...
Carbohydrate-binding module
"Mutations in the gal83 glycogen-binding domain activate the snf1/gal83 kinase pathway by a glycogen-independent mechanism". ... 6-glucosidic linkages of glycogen; and pullulanase is a starch-debranching enzyme. CBM48 binds glycogen. Carbohydrate-binding ... Armstrong, C. G.; Doherty, M. J.; Cohen, P. T. (1998). "Identification of the separate domains in the hepatic glycogen- ... Isoamylase hydrolyses 1,6-alpha-D-glucosidic branch linkages in glycogen, amylopectin and dextrin; 1,4-alpha-glucan branching ...
Fight-or-flight response
"PATHWAYS: GLYCOGEN & GLUCOSE". Washington University in St. Louis. "HOW CELLS COMMUNICATE DURING THE FIGHT OR FLIGHT RESPONSE ... particularly fat and glycogen) for muscular action[citation needed] Dilation of blood vessels for muscles[citation needed] ...
Robert Frederick Mahler
Mahler, Robert (1969). "Glycogen storage diseases". J Clin Pathol Suppl (Assoc Clin Pathol). 2: 32-41. doi:10.1136/jcp.22.Suppl ...
Metabolism
The metabolism of glycogen is controlled by activity of phosphorylase, the enzyme that breaks down glycogen, and glycogen ... Insulin causes glycogen synthesis by activating protein phosphatases and producing a decrease in the phosphorylation of these ... Newgard CB, Brady MJ, O'Doherty RM, Saltiel AR (December 2000). "Organizing glucose disposal: emerging roles of the glycogen ... These enzymes are regulated in a reciprocal fashion, with phosphorylation inhibiting glycogen synthase, but activating ...
Acid alpha-glucosidase
... that helps to break down glycogen in the lysosome. It is functionally similar to glycogen debranching enzyme, but is on a ... Defects in this gene are the cause of glycogen storage disease II, also known as Pompe disease, which is an autosomal recessive ... GeneReview/NIH/UW entry on Glycogen Storage Disease Type II (Pompe Disease) Human GAA genome location and GAA gene details page ... Errors in this gene cause glycogen storage disease type II (Pompe disease). This gene encodes lysosomal alpha-glucosidase, ...
Starch synthase
Greenberg E; Preiss J (1965). "Biosynthesis of bacterial glycogen. II. Purification and properties of the adenosine ... diphosphoglucose:glycogen transglucosylase of arthrobacter species NRRL B1973". J. Biol. Chem. 240: 2341-2348. PMID 14304835. ...
Phosphofructokinase deficiency
A diagnosis can be made through a muscle biopsy that shows excess glycogen accumulation. Glycogen deposits in the muscle are a ... "Glycogen Storage Disease Type VII". Genetics Home Reference. US National Library of Medicine. Toscano A, Musumeci O (October ... Unlike most other glycogen storage diseases, it directly affects glycolysis. The mutation impairs the ability of ... "Glycogen Storage Disease Type VII". Rare Disease Database. National Organization for Rare Disorders. Swoboda, Kathryn; Specht, ...
Apostomatida
Effect of glycogen" (PDF). Biological Bulletin. 133 (2): 310-6. "Apostome". Encyclopædia Britannica. v t e (Articles with short ...