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*  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 ... Glycogen phosphorylase can act only on linear chains of glycogen (α1-4 glycosidic linkage). Its work will immediately come to a ... Glycogen phosphorylase is one of the phosphorylase enzymes (EC 2.4.1.1). Glycogen phosphorylase catalyzes the rate-limiting ...
*  Glycogen storage disease type I
Glycogen-Storage Disease Type I at eMedicine The Association for Glycogen Storage Disease: Type I Glycogen Storage Disease Type ... Glycogen storage disease type I (GSD I) or von Gierke disease, is the most common of the glycogen storage diseases. This ... 1993). "Glycogen Storage Disease Type I". PMID 20301489. Online Mendelian Inheritance in Man (OMIM) Glycogen Storage Disease Ib ... 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
GSD type 0: Although glycogen synthase deficiency does not result in storage of extra glycogen in the liver, it is often ... Glycogen Metabolism themedicalbiochemistrypage.org eMedicine Specialties > Glycogen-Storage Disease Type I Author: Karl S Roth ... Updated: Aug 31, 2009 The Association for Glycogen Storage Disease > Type I Glycogen Storage Disease Type I GSD Archived 2010- ... is a metabolic disorder caused by enzyme deficiencies affecting either glycogen synthesis, glycogen breakdown or glycolysis ( ...
*  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 ... Glycogen debranching enzymes assist phosphorylase, the primary enzyme involved in glycogen breakdown, mobilize glycogen stores ... In GSD III glycogen breakdown is incomplete and there is accumulation of abnormal glycogen with short outer branches. Most ...
*  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 Branching Enzyme Deficiency (GBED) VetGen: Veterinary Genetics Services: Glycogen Branching Enzyme Deficiency (GBED). ...
*  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 ...
*  Pullulanase
... will hydrolytically cleave pullulan (alpha-glucan polysaccharides). Lee, E.Y.C.; Whelan, W.J. (1972). "Glycogen and ... Bender, H.; Wallenfels, K. (1966). "Pullulanase (an amylopectin and glycogen debranching enzyme) from Aerobacter aerogenes". ...
*  Glycogenin-1
... glycogen synthase (GS), and for its degradation, glycogen phosphorylase (GF). Glycogenin is the initiator of the glycogen ... It also plays a role in glycogen metabolism regulation and in the maximal glycogen levels attaintment in skeletal muscle. ... Glucose Glycogen Glycogen synthase Glycogenin Gene Mutation GRCh38: Ensembl release 89: ENSG00000163754 - Ensembl, May 2017 ... this tissue contains three quarters of the corporal glycogen. On the one hand, the function of the liver glycogen is to ...
*  Glycogen storage disease type II
"Type II Glycogen Storage Disease". The Association for Glycogen Storage Disease. Archived from the original on 23 June 2012. ... It is the only glycogen storage disease with a defect in lysosomal metabolism, and the first glycogen storage disease to be ... 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
Due to the way glycogen is synthesised, every glycogen granule has at its core a glycogenin protein. Glycogen in muscle, liver ... Glycogen is cleaved from the nonreducing ends of the chain by the enzyme glycogen phosphorylase to produce monomers of glucose- ... Glycogen phosphorylase is the primary enzyme of glycogen breakdown. For the next 8-12 hours, glucose derived from liver ... Glycogen synthesis is, unlike its breakdown, endergonic-it requires the input of energy. Energy for glycogen synthesis comes ...
*  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 kinase
... is an enzyme. Types include: GSK1 GSK2 GSK-3 GSK3A GSK3B Glycogen synthase kinase at the US National ...
*  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 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-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 ... In enzymology, a [glycogen-synthase-D] phosphatase (EC 3.1.3.42) is an enzyme that catalyzes the chemical reaction [glycogen- ... the two substrates of this enzyme are glycogen-synthase D and H2O, whereas its two products are glycogen-synthase I and ...
*  Glycogen storage disease type IX
... is a hereditary deficiency of glycogen phosphorylase kinase B that affects the liver and ... Glycogen storage disease "Glycogen storage disease type IX". Genetics Home Reference. Retrieved 2016-08-06. Goldstein, Jennifer ... glycogen storage disease IXa1 and glycogen storage disease IXa2 that affect the liver of an individual. Mutations in PHKA2 have ... Glycogen storage disease type IX can be inherited via: X-linked recessive inheritance due to mutations at either PHKA1 or the ...
*  Glycogen storage disease type IV
... , also known as Anderson's Disease, is a form of glycogen storage disease, which is caused by ... 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 ... Glycogen Storage Disease Type IV is autosomal recessive, which means each parent has a mutant copy of the gene but show no ...
*  Glycogen storage disease type XI
... is a form of glycogen storage disease. It is also known as "Fanconi-Bickel syndrome", for ... The syndrome results in hepatomegaly secondary to glycogen accumulation, glucose and galactose intolerance, fasting ...
*  Glycogen storage disease type VI
... (GSD VI) is a type of glycogen storage disease caused by a deficiency in liver glycogen ... Phosphorylase kinase Glycogen-Storage Disease Type VI at eMedicine Hers HG (1959). "[Enzymatic studies of hepatic fragments; ... The scope of GSD VI now also includes glycogen storage disease type VIII, IX (caused by phosphorylase b kinase deficiency) and ... http://ghr.nlm.nih.gov/condition/glycogen-storage-disease-type-vi GeneReview/NIH/UW entry on Glycogen Storage Disease Type VI. ...
*  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 ... The gene is responsible for creating glycogen debranching enzyme, which in turn helps in glycogen decomposition. In terms of ... Glycogen is a molecule the body uses to store carbohydrate energy. Symptoms of GSD-III are caused by a deficiency of the enzyme ...
*  Glycogen storage disease type 0
"Glycogen-Storage Disease Type 0" "Orphanet: Glycogen storage disease due to hepatic glycogen synthase deficiency". www.orpha. ... e condition glycogen storage disease type 0 to be considered, they are: Glycogen storage disease due to liver glycogen synthase ... Glycogen storage disease type 0 is a disease characterized by a deficiency in the glycogen synthase enzyme (GYS). Although ... Glycogen synthetase catalyzes the rate-limiting reaction for glycogen synthesis in the liver by transferring glucose units from ...
*  Glycogen storage disease type V
... (GSD-V) is a metabolic disorder, more specifically a glycogen storage disease, caused by a ... The Association for Glycogen Storage Disease The Association for Glycogen Storage Disease (UK) McArdle information McArdle ... In some cases, acid-Schiff stained glycogen can be seen with microscopy. Genetic sequencing of the PYGM gene (which codes for ... Its incidence is reported as 1 in 100,000, approximately the same as glycogen storage disease type I. The disease was first ...
*  DMOZ - Health: Conditions and Diseases: Neurological Disorders: Brain Diseases: Glycogen Storage Disease Type II
Large amounts of glycogen accumulate in the lysomes of skeletal muscle ( muscle, skeletal); heart; liver; spinal cord; and ... An autosomal recessively inherited glycogen storage disease caused by glucan 1,4-Alpa-Glucosidase Definciency. ... "Health ... Glycogen Storage Disease Type II" search on: AOL - Ask - Bing - DuckDuckGo - Gigablast - Google - ixquick - Yahoo - ... Health Conditions and Diseases Neurological Disorders Brain Diseases Glycogen Storage Disease Type II 4 ...
*  DMOZ - Health: Conditions and Diseases: Nutritional and Metabolic Disorders: Inherited: Glycogen Storage Disease Type I
The Association For Glycogen Storage Disease The Association for Glycogen Storage Disease is a parent and patient oriented ... "Health ... Glycogen Storage Disease Type I" search on: AOL - Ask - Bing - DuckDuckGo - Gigablast - Google - ixquick - Yahoo - ... Health Conditions and Diseases Nutritional and Metabolic Disorders Inherited Glycogen Storage Disease Type I 3 ... The Children's Fund for Glycogen Storage Disease Research This not-for-profit foundation has been established to benefit ...
*  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 ...