LiverSynthaseSynthesisDeficiencyActivates glycogen phosphorylaseGluconeogenesisDiseasesMuscleKinaseMutationsInhibits glycogenProteinBreakdownAccumulationProteinsMetabolismHypoglycemiaPhosphorylationHepaticPyridoxal phosphateGlycogenolysisBranched polymer of glucoseMusclesAbnormalExcessMetabolicDisordersMoleculeAllostericInsulin resistanceCatalyzesGlycogenesisSubunitsGlycosidicAdditionallyAmino acidsCatalyticResiduesConvertsPrecursorCatalyzed by distiMechanismStoresEnergyMitochondrialDisease typePatients
Liver34
- In mammals, the major isozymes of glycogen phosphorylase are found in muscle, liver, and brain. (wikipedia.org)
- The different forms of the condition can affect glycogen breakdown in liver cells or muscle cells or sometimes both. (medlineplus.gov)
- At least two different versions of phosphorylase b kinase are formed from the subunits: one is most abundant in liver cells and the other in muscle cells. (medlineplus.gov)
- Whether in the liver or the muscles, phosphorylase b kinase plays an important role in providing energy for cells. (medlineplus.gov)
- Glucose is stored in muscle and liver cells in a form called glycogen. (medlineplus.gov)
- Although the effects of gene mutations on the respective protein subunits are unknown, mutations in the PHKA1 , PHKA2 , PHKB , and PHKG2 genes reduce the activity of phosphorylase b kinase in liver or muscle cells and in blood cells. (medlineplus.gov)
- Glycogen accumulation in the liver leads to hepatomegaly, and the liver's inability to break down glycogen for glucose contributes to hypoglycemia and ketosis. (medlineplus.gov)
- Glucagon inhibits the production of insulin, resulting in decreased glucose storage in the liver. (satietytoday.com)
- Hepatic phosphorylase deficiency or deficiency of other enzymes that form a cascade necessary for liver phosphorylase activation cause the disease. (medscape.com)
- Disorders that result from defective glycogenolysis in the liver lead to hypoglycemia within a few hours of fasting. (medscape.com)
- 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)
- Together with phosphorylases , debranching enzymes mobilize glucose reserves from glycogen deposits in the muscles and liver. (wikidoc.org)
- Glycogen breakdown is highly regulated in the body, especially in the liver , by various hormones including insulin and glucagon , to maintain a homeostatic balance of blood-glucose levels. (wikidoc.org)
- This enzyme converts the glucose absorbed by the liver into either of the two: Glycogen - in the process of glycogenesis or into pyruvate which will eventually be converted into energy, in the process of glycolysis. (dnx.news)
- Now in a fasting state (hypoglycemia) in which the blood lacks glucose, Glucagon binds to its receptor in the liver and activates another receptor called the "g-protein coupled receptor" and eventually along the way this process lead to the activation of protein kinase A (PKA). (dnx.news)
- The liver at this state has a storage of glycogen due to the previous glycogenesis processes helped by insulin. (dnx.news)
- Different parts of the muscles and the liver act as storage units for glycogen. (xcode.life)
- Now, some branching enzymes add branches to Glycogen, which the liver then stores as a reservoir of energy. (xcode.life)
- Butyrate, end-product of intestinal fermentation, is known to impair oxidative phosphorylation in rat liver and could disturb glycogen synthesis depending on the ATP supplied by mitochondrial oxidative phosphorylation and cytosolic glycolysis. (biomedcentral.com)
- A net linear flux of glycogen synthesis (~11.10 ± 0.60 μmol glucosyl units.h -1 .g -1 liver wet weight) occurred until the 6th hr post-feeding in both groups, whereas butyrate delayed it until the 8th hr. (biomedcentral.com)
- In liver from 48 hr-starved rats, the energy need for net glycogen synthesis from exogenous glucose corresponds to ~50% of basal mitochondrial ATP turnover. (biomedcentral.com)
- The liver is the main site of glycogen contribution to the regulation of blood glucose. (biomedcentral.com)
- 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)
- Reproductive system: these are composed of smooth muscle and express either brain or liver uterus, testis, probably glycogen phosphorylase (or both). (integralbiosciences.com)
- Glycogenesis is the process by which glucose molecules converted into glycogen for storage in the liver and muscles. (classicbiotech.com)
- Glycogenesis is a vital metabolic process that occurs in the liver and muscles, allowing the body to store excess glucose as glycogen for future energy needs. (classicbiotech.com)
- Glycogenesis occurs primarily in the liver and muscles, where glucose converted into glycogen through a series of enzymatic reactions. (classicbiotech.com)
- Glycogen storage disease type III (GSD III) is characterized by variable liver, cardiac muscle, and skeletal muscle involvement. (nih.gov)
- Background: Glycogen-storage disease type VI (GSD VI) is an autosomal recessive disorder due to deficiency of the liver isoform of phosphorylase resulting in abnormal accumulation of glycogen. (eurospe.org)
- Glycogenolysis: Free blood glucose can be derived from the liver stores of glycogen. (online-sciences.com)
- 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)
- 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)
- Excess glucose is stored as glycogen in our skeletal muscles and our liver. (iamgsd.org)
- It follows, therefore, that defects at the level of the β-cell, muscle and liver can lead to the development of glucose intolerance and diabetes mellitus. (justia.com)
Synthase7
- Phosphorylation of the second enzyme, glycogen synthase (GS), inhibits its ability to form glycogen from glucose. (lumenlearning.com)
- Glycogen synthase: towards a minimum catalytic unit? (concordia.ca)
- Another enzyme, glycogen synthase, helps in the conversion of glucose into Glycogen. (xcode.life)
- The activated glucose molecule, UDP-glucose, is added to the growing glycogen chain by the enzyme glycogen synthase. (classicbiotech.com)
- The elongation of the glycogen chain occurs through the action of the enzyme glycogen synthase. (classicbiotech.com)
- Glycogen synthase catalyzes the transfer of UDP-glucose to the growing glycogen chain, forming an α-1,4-glycosidic bond. (classicbiotech.com)
- It also activates glycogen synthase, the enzyme responsible for glycogen synthesis, by dephosphorylating it. (classicbiotech.com)
Synthesis8
- In this manner, a muscle cell obtains a ready pool of glucose by activating its formation via glycogen degradation and by inhibiting the use of glucose to form glycogen, thus preventing a futile cycle of glycogen degradation and synthesis. (lumenlearning.com)
- Protein targeting to glycogen (PTG) is a scaffolding protein that targets protein phosphatase 1α (PP1α) to glycogen, and links it to enzymes involved in glycogen synthesis and degradation. (jci.org)
- These data suggest that PTG plays a critical role in glycogen synthesis and is necessary to maintain the appropriate metabolic balance for the partitioning of fuel substrates between glycogen and lipid. (jci.org)
- A linear correlation between total ATP and glycogen contents was obtained (r 2 = 0.99) only during net glycogen synthesis. (biomedcentral.com)
- Glycogen synthesis, which is located in cytosol, depends on the UTP supply and hence on ATP supply resulting from both mitochondrial oxidative phosphorylation and cytosolic glycolysis. (biomedcentral.com)
- For example, in familial hypercholesterolemia, enzymes do not receive the signals that typically inhibit cholesterol synthesis, so that excessive production of cholesterol occurs, leading to early coronary vascular disease and strokes in patients. (newworldencyclopedia.org)
- This step is crucial as UDP-glucose serves as the precursor for glycogen synthesis. (classicbiotech.com)
- There are many numbered and named types, all of which are caused by deficiencies of enzymes involved in glycogen synthesis or. (msdmanuals.com)
Deficiency7
- Its clinical presentation clearly differs from other forms of GSD, because it is caused by the deficiency of the lysosomal enzyme, alpha-1,4-glucosidase, leading to the pathologic accumulation of normally structured glycogen within the lysosomes of most tissues, differs Three forms of the disease exist: infantile-onset, late-onset juvenile and adult onset. (medscape.com)
- It is an autosomal recessive disorder in which there is an AGL gene mutations which causes deficiency in glycogen debranchinging enzyme and limited storage of dextrin. (medscape.com)
- GSD type IV, also known as amylopectinosis, Glycogen Branching enzyme deficiency (GBE) or Andersen disease, is a rare disease that leads to early death. (medscape.com)
- Muscle phosphorylase deficiency adversely affecting the glycolytic pathway in skeletal musculature causes GSD type V. Like other forms of GSD, McArdle disease is heterogeneous. (medscape.com)
- DiMauro SHartlage PL Fatal infantile form of muscle phosphorylase deficiency. (jamanetwork.com)
- The main cause is either insulin deficiency or insulin resistance, which leads to the accumulation of glucose in the blood and hence elevated blood glucose levels. (online-sciences.com)
- Individuals with a GSD either have an absence or deficiency of one of the enzymes that are responsible for making, storing or using glycogen. (iamgsd.org)
Activates glycogen phosphorylase2
- Glucagon also activates glycogen phosphorylase and debranching enzymes, which break down stored glucose to glucose. (satietytoday.com)
- The first enzyme promotes the degradation of glycogen by activating intermediate glycogen phosphorylase kinase (GPK) that in turn activates glycogen phosphorylase (GP) that catabolizes glycogen into glucose. (lumenlearning.com)
Gluconeogenesis3
- Fasting leads to changes in several metabolic pathways, including gluconeogenesis and glycogenolysis. (satietytoday.com)
- Akt also stimulates the production of glycogen and inhibits gluconeogenesis. (encyclopedia.pub)
- In contrast to glycolysis that creates glucose from stored glycogen, gluconeogenesis creates glucose through the help of protein kinase A from non-carbohydrate products, such as glycerol, glucogenic amino acids and even lactate. (dnx.news)
Diseases9
- [1] When glycogen breakdown is compromised by mutations in the glycogen debranching enzyme, metabolic diseases such as Glycogen storage disease type III can result. (wikidoc.org)
- Diabetic nephropathy (DN), one of the most common complications of Diabetes Mellitus, is the leading cause of end-stage renal diseases worldwide. (bvsalud.org)
- When these enzymes don't function optimally, it leads to a spectrum of diseases. (xcode.life)
- This could lead to a range of different symptoms depending on the type of diseases. (xcode.life)
- What causes Glycogen Storage Diseases? (xcode.life)
- The three main categories of disease include the mitochondrial myopathies, fatty acid oxidation defects (FAODs), and glycogen storage diseases (GSDs). (essayempire.com)
- Some severe diseases, such as many of the lipid storage diseases, currently have no effective therapy. (newworldencyclopedia.org)
- DiMauro SServidei STsujino S Disorders of carbohydrate metabolism: glycogen storage diseases. (jamanetwork.com)
- Glycogen Storage Diseases Glycogen storage diseases are carbohydrate metabolism disorders. (msdmanuals.com)
Muscle8
- 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)
- The allosteric site of AMP binding on muscle isoforms of glycogen phosphorylase are close to the subunit interface just like Ser14. (wikipedia.org)
- The activation of β-adrenergic receptors in muscle cells by adrenaline leads to an increase in cyclic AMP (cAMP) inside the cell. (lumenlearning.com)
- Burke JHwang PAnderson LLebo RGorin FFletterick R Intron/exon structure of the human gene for the muscle isozyme of glycogen phosphorylase. (jamanetwork.com)
- Bartram CEdwards RHTClague JBeynon RJ McArdle's disease: a nonsense mutation in exon 1 of the muscle glycogen phosphorylase gene explains some but not all cases. (jamanetwork.com)
- IamGSD is focused on "McArdle Disease and related disorders of muscle glycogen where physical activity intolerance is the major symptom. (iamgsd.org)
- Muscle glycogen storage disease is when our muscles cannot convert their glycogen back into glucose to power our muscles. (iamgsd.org)
- Pyridoxal phosphate is a cofactor for glycogen phosphorylase, which releases glucose-1-phosphate from glycogen and provides additional glucose when needed, such as in exercising muscle. (savvybeverage.com.au)
Kinase5
- These genes provide instructions for making pieces (subunits) of an enzyme called phosphorylase b kinase. (medlineplus.gov)
- The PHKA1 and PHKA2 genes provide instructions for making alpha subunits of phosphorylase b kinase. (medlineplus.gov)
- Phosphorylase b kinase turns on (activates) the enzyme that breaks down glycogen. (medlineplus.gov)
- Protein kinase A activates an enzyme called glycogen phosphorylase to break down this glycogen into glucose in a process called glycogenolysis. (dnx.news)
- leads to modifications in mitogen turned on proteins kinase (MAPK) signaling pathways as the 106-126 peptide activates development aspect related signaling pathways including vascular endothelial development aspect (VEGF) signaling as well as the phosphoinositide-3 kinase (PI3K) pathway. (fabretp.org)
Mutations2
- For example, the mutations responsible for a number of autosomal recessive conditions have been found, including myo-neuro-gastrointestinal encephalomyopathy (MNGIE, thymidine phosphorylase), complex I (NDUF) and IV (SURF-1) Leigh's disease, mtDNA depletion (dGuOK, TK) and some forms of mtDNA deletion syndromes (polymerase gamma, twinkle, ANT). (essayempire.com)
- Various autosomal dominant heterozygous INSR mutations leading to hyperinsulinemic hypoglycemia (HH) have been de. (eurospe.org)
Inhibits glycogen1
- Additionally, insulin inhibits glycogen phosphorylase, the enzyme responsible for glycogen breakdown, further promoting glycogenesis. (classicbiotech.com)
Protein7
- Glycogen phosphorylase is also studied as a model protein regulated by both reversible phosphorylation and allosteric effects. (wikipedia.org)
- This lack of easy access of the catalytic site to the surface is significant in that it makes the protein activity highly susceptible to regulation, as small allosteric effects could greatly increase the relative access of glycogen to the site. (wikipedia.org)
- Once these glycogen stores are depleted, the body uses stored protein and fatty acids to supply energy. (satietytoday.com)
- Rac1 protein regulates glycogen phosphorylase activation and controls interleukin (IL)-2-dependent T cell proliferation. (concordia.ca)
- In this study, effect of foliar application of micronutrient fertilizers included iron, zinc, copper and manganese were investigated on the activity of antioxidant enzymes, amount of antioxidant non-enzymatic compounds and storage-macromolecules (protein, glycogen and triglyceride) of the third and fourth instar larvae of H. variegata fed on M. persicae. (ac.ir)
- Results showed that the higher amount of protein, glycogen and triglyceride of the third and the fourth instar larvae of H. variegata were obtained on micronutrient fertilizers. (ac.ir)
- Positive Darwinian natural selection can bring about an increase in either component, and negative or stabilizing selection in protein evolution can lead to decreases. (pharmaceuticalintelligence.com)
Breakdown6
- A lack of glycogen breakdown interferes with the normal function of the affected tissue. (medlineplus.gov)
- Reduction of this enzyme's function impairs glycogen breakdown. (medlineplus.gov)
- A debranching enzyme is a molecule that helps facilitate the breakdown of glycogen , which serves as a store of glucose in the body, through glucosyltransferase and glucosidase activity. (wikidoc.org)
- Together with phosphorylase , glycogen debranching enzymes function in glycogen breakdown and glucose mobilization. (wikidoc.org)
- Glycogen debranching enzymes assist phosphorylase, the primary enzyme involved in glycogen breakdown , mobilize glycogen stores. (wikidoc.org)
- This branching allows for multiple sites of glucose release during glycogen breakdown. (classicbiotech.com)
Accumulation2
- An essential pathologic finding is the accumulation of normally structured glycogen in most tissues. (medscape.com)
- The increase of superoxide dismutase activity can indicate the occurrence of oxidative stress in the body of the third and the fourth instar larvae of predator H. variegata could be assigned to the accumulation of H2O2 in the body of M. persicae under the influence of the defensive secondary metabolites, which led to increase the activity of catalase and peroxidase. (ac.ir)
Proteins3
- This leads to the conversion of glucose into ketones, which satisfy the brain's energy needs while preserving the proteins in our cells. (satietytoday.com)
- Proteins that catalyze both functions are referred to as glycogen debranching enzymes (GDEs). (wikidoc.org)
- For this process to take place, some special proteins called enzymes (biocatalysts) aid the formation and deformation of glycogen. (xcode.life)
Metabolism4
- The contribution of energetic metabolism to glycogen metabolism was estimated. (biomedcentral.com)
- Butyrate, known to reduce oxidative phosphorylation yield and to induce a glucose-sparing effect, delayed the transient increase in mitochondrial ATP turnover and hence energy contribution to glycogen metabolism. (biomedcentral.com)
- Gut microbiota clearly affect the host nutritional metabolism with consequences for energy storage, which implies mechanistic interactions between events occurring in the colon and the regulation of energy metabolism [ 3 ]. (biomedcentral.com)
- Scheme of glycogen metabolism and glycolysis. (iamgsd.org)
Hypoglycemia1
- Hypoglycemia is of concern and may lead to hypoglycemic seizures. (medscape.com)
Phosphorylation1
- 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)
Hepatic1
- Glucagon is the primary hormone regulating hepatic glucose production, and when it is suppressed, it leads to a near-total suppression of glucose output in the bloodstream. (satietytoday.com)
Pyridoxal phosphate2
- Glycogen phosphorylase has a pyridoxal phosphate (PLP, derived from Vitamin B6) at each catalytic site. (wikipedia.org)
- There are several different mechanisms that lead to an increased requirement for pyridoxine and/or pyridoxal phosphate. (savvybeverage.com.au)
Glycogenolysis1
- 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)
Branched polymer of glucose1
- Glycogen is a branched polymer of glucose units that is formed in times of nutrient sufficiency and it is utilized in times of need. (concordia.ca)
Muscles1
- This stored fuel (glycogen) is then used for energy in in the skeletal muscles throughout our bodies. (iamgsd.org)
Abnormal1
- Glycogen deposited in these organs has an abnormal structure. (medscape.com)
Excess2
- Note that your body converts excess glucose to glycogen for short-term storage. (lumenlearning.com)
- The excess glucose that is converted into glycogen may be used by the immune system when nutrients are low. (concordia.ca)
Metabolic1
- Glycogen storage disease (GSD) type I, also known as von Gierke disease, is a group of inherited autosomal recessive metabolic disorders of the glucose-6- phosphatase system which helps maintain glucose homeostasis. (medscape.com)
Disorders1
- The disease can lead to various health complications, including disorders of the cardiovascular system, eyes , kidneys , and nerves, Untreated or poorly treated diabetes accounts for approximately 1.5 million deaths every year. (online-sciences.com)
Molecule4
- Glycogen is left with one fewer glucose molecule, and the free glucose molecule is in the form of glucose-1-phosphate. (wikipedia.org)
- The protonated oxygen now represents a good leaving group, and the glycogen chain is separated from the terminal glycogen in an SN1 fashion, resulting in the formation of a glucose molecule with a secondary carbocation at the 1 position. (wikipedia.org)
- Finally, the deprotonated inorganic phosphate acts as a nucleophile and bonds with the carbocation, resulting in the formation of glucose-1-phosphate and a glycogen chain shortened by one glucose molecule. (wikipedia.org)
- Glycogen Phosphorylase and glycogen debranching enzymes help in unraveling the molecule to release glucose and expend energy. (xcode.life)
Allosteric2
- 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)
- Hudson JWGolding GBCrerar MM Evolution of allosteric control in glycogen phosphorylase. (jamanetwork.com)
Insulin resistance1
- With time, however, the beta-cell fails to maintain its high rate of insulin secretion and the insulin resistance leads to the development of impaired glucose tolerance and eventually overt diabetes mellitus. (justia.com)
Catalyzes2
- This enzyme catalyzes the formation of α-1,4-glycosidic bonds between glucose molecules, extending the glycogen chain. (classicbiotech.com)
- The enzyme glycogen branching enzyme (GBE) catalyzes the transfer of a segment of the glycogen chain to a nearby glucose residue, forming an α-1,6-glycosidic bond. (classicbiotech.com)
Glycogenesis1
- The process of glycogenesis is tightly regulated to ensure proper glucose storage and release when needed. (classicbiotech.com)
Subunits1
- 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)
Glycosidic3
- Glycogen phosphorylase can act only on linear chains of glycogen (α1-4 glycosidic linkage). (wikipedia.org)
- Phosphorylase can only cleave α-1,4- glycosidic bond between adjacent glucose molecules in glycogen but branches exist as α-1,6 linkages. (wikidoc.org)
- This enzyme transfers a segment of the glycogen chain to form an α-1,6-glycosidic bond, creating a branch point. (classicbiotech.com)
Additionally2
- Additionally, it leads to a decrease in PEPCK expression, which plays a role in the conversion of glycogen to glucose. (satietytoday.com)
- Additionally, when the amount of nutrients was lowered, less glycogen was stored in PBMCs. (concordia.ca)
Amino acids1
- Glucose and several amino acids stimulate insulin secretion under physiologic conditions, and the sequence of events leading to insulin secretion is well delineated. (medscape.com)
Catalytic1
- This crevice connects the glycogen storage site to the active, catalytic site. (wikipedia.org)
Residues4
- Its work will immediately come to a halt four residues away from α1-6 branch (which are exceedingly common in glycogen). (wikipedia.org)
- When phosphorylase has digested a glycogen branch down to four glucose residues, it will not remove further residues. (wikidoc.org)
- because 1 in 10 residues is branched, cleavage by phosphorylase alone would not be sufficient in mobilizing glycogen stores. (wikidoc.org)
- 4-α-D-glucanotransferase ( EC 2.4.1.25 ), or glucosyltransferase , transfers three glucose residues from the four-residue glycogen branch to a nearby branch. (wikidoc.org)
Converts1
- Thus the debranching enzymes, transferase and α-1,6- glucosidase converts the branched glycogen structure into a linear one, paving the way for further cleavage by phosphorylase. (wikidoc.org)
Precursor1
- Today's paper asserts a provocative hypothesis that autoimmune pathomechanisms influencing the bloodCbrain hurdle (BBB) or bloodCspinal hurdle (BSB) may predispose the BBB/BSB to leakiness and become a precursor to extra autoimmune events leading to neuroinflammatory or neurodegenerative procedures, compounded on the genetically susceptible record or contact with environmental reasons possibly. (euromed2016.com)
Catalyzed by disti1
- When glucosyltransferase and glucosidase are catalyzed by distinct enzymes, "glycogen debranching enzyme" usually refers to the glucosidase enzyme . (wikidoc.org)
Mechanism2
- This intricate mechanism involves a series of enzymatic reactions that convert glucose molecules into glycogen, ensuring a steady supply of energy during periods of fasting or increased physical activity. (classicbiotech.com)
- From these initiatives a number of physiological features of PrPC have already been recommended including: neural security,3 copper fat burning capacity,4 long-term storage Valaciclovir development,5 and bone tissue marrow renewal.6 There is certainly equal uncertainty from the mechanism of PrPSc pathology. (fabretp.org)
Stores2
- In the presence of high glucose, lymphocytes build up glycogen stores, but the fate of this content is not very well understood. (concordia.ca)
- This study demonstrated that activated PBMCs contain more glycogen stores as compared to non-activated cells. (concordia.ca)
Energy6
- Glycogen can be broken down rapidly when glucose is needed, for instance to maintain normal levels of glucose in the blood between meals or for energy during exercise. (medlineplus.gov)
- As a result, glycogen accumulates in and damages cells, and glucose is not available for energy. (medlineplus.gov)
- When energy is needed, glycogen is quickly reconverted to glucose. (lumenlearning.com)
- Our body uses glycogen, a complex sugar compound, as a fundamental storing unit of energy. (xcode.life)
- Metabolizing glycogen, to break it down into glucose, provides the instant energy we need. (xcode.life)
- As we need to conserve some of this energy, these glucose molecules are combined back into glycogen. (xcode.life)
Mitochondrial1
- The evidence of a late and transient increase in mitochondrial ATP turnover reflects an energetic need, probably linked to a glycogen cycling. (biomedcentral.com)
Disease type1
- Glycogen storage disease type IX (also known as GSD IX) is a condition caused by the inability to break down a complex sugar called glycogen. (medlineplus.gov)
Patients1
- We are a patient-led international group encouraging efforts by research and medical professionals, national support groups and individual patients worldwide. (iamgsd.org)