A class of glucosyltransferases that catalyzes the degradation of storage polysaccharides, such as glucose polymers, by phosphorolysis in animals (GLYCOGEN PHOSPHORYLASE) and in plants (STARCH PHOSPHORYLASE).
An isoenzyme of GLYCOGEN PHOSPHORYLASE that catalyzes the degradation of GLYCOGEN in muscle. Mutation of the gene coding this enzyme is the cause of McArdle disease (GLYCOGEN STORAGE DISEASE TYPE V).
An enzyme that catalyzes the degradation of GLYCOGEN in animals by releasing glucose-1-phosphate from the terminal alpha-1,4-glycosidic bond. This enzyme exists in two forms: an active phosphorylated form ( PHOSPHORYLASE A) and an inactive un-phosphorylated form (PHOSPHORYLASE B). Both a and b forms of phosphorylase exist as homodimers. In mammals, the major isozymes of glycogen phosphorylase are found in muscle, liver and brain tissue.
The inactive form of GLYCOGEN PHOSPHORYLASE that is converted to the active form PHOSPHORYLASE A via phosphorylation by PHOSPHORYLASE KINASE and ATP.
An isoenzyme of GLYCOGEN PHOSPHORYLASE that catalyzes the degradation of GLYCOGEN in liver tissue. Mutation of the gene coding this enzyme on chromosome 14 is the cause of GLYCOGEN STORAGE DISEASE TYPE VI.
Contractile tissue that produces movement in animals.
The active form of GLYCOGEN PHOSPHORYLASE that is derived from the phosphorylation of PHOSPHORYLASE B. Phosphorylase a is deactivated via hydrolysis of phosphoserine by PHOSPHORYLASE PHOSPHATASE to form PHOSPHORYLASE B.
An enzyme that catalyzes the transfer of D-glucose from UDPglucose into 1,4-alpha-D-glucosyl chains. EC 2.4.1.11.
An enzyme that catalyzes the conversion of ATP and PHOSPHORYLASE B to ADP and PHOSPHORYLASE A.
Enzymes that catalyze the transfer of glucose from a nucleoside diphosphate glucose to an acceptor molecule which is frequently another carbohydrate. EC 2.4.1.-.
An isoenzyme of GLYCOGEN PHOSPHORYLASE that catalyzes the degradation of GLYCOGEN in brain tissue.
Glycogen stored in the liver. (Dorland, 28th ed)
An enzyme that catalyzes the reaction between a purine nucleoside and orthophosphate to form a free purine plus ribose-5-phosphate. EC 2.4.2.1.
An enzyme that catalyzes the transfer of ribose from uridine to orthophosphate, forming uracil and ribose 1-phosphate.
Five-carbon furanose sugars in which the OXYGEN is replaced by a NITROGEN atom.
A subtype of striated muscle, attached by TENDONS to the SKELETON. Skeletal muscles are innervated and their movement can be consciously controlled. They are also called voluntary muscles.
Adenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position.
An enzyme that catalyzes the transfer of 2-deoxy-D-ribose from THYMIDINE to orthophosphate, thereby liberating thymidine.
A key intermediate in carbohydrate metabolism. Serves as a precursor of glycogen, can be metabolized into UDPgalactose and UDPglucuronic acid which can then be incorporated into polysaccharides as galactose and glucuronic acid. Also serves as a precursor of sucrose lipopolysaccharides, and glycosphingolipids.
The species Oryctolagus cuniculus, in the family Leporidae, order LAGOMORPHA. Rabbits are born in burrows, furless, and with eyes and ears closed. In contrast with HARES, rabbits have 22 chromosome pairs.
The protein constituents of muscle, the major ones being ACTINS and MYOSINS. More than a dozen accessory proteins exist including TROPONIN; TROPOMYOSIN; and DYSTROPHIN.
An enzyme that deactivates glycogen phosphorylase a by releasing inorganic phosphate and phosphorylase b, the inactive form. EC 3.1.3.17.
A hepatic GLYCOGEN STORAGE DISEASE in which there is an apparent deficiency of hepatic phosphorylase (GLYCOGEN PHOSPHORYLASE, LIVER FORM) activity.
A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement.
A group of inherited metabolic disorders involving the enzymes responsible for the synthesis and degradation of glycogen. In some patients, prominent liver involvement is presented. In others, more generalized storage of glycogen occurs, sometimes with prominent cardiac involvement.
An ester of glucose with phosphoric acid, made in the course of glucose metabolism by mammalian and other cells. It is a normal constituent of resting muscle and probably is in constant equilibrium with fructose-6-phosphate. (Stedman, 26th ed)
Conjugated protein-carbohydrate compounds including mucins, mucoid, and amyloid glycoproteins.
A glycogen synthase kinase that was originally described as a key enzyme involved in glycogen metabolism. It regulates a diverse array of functions such as CELL DIVISION, microtubule function and APOPTOSIS.
A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.
Glycogenosis due to muscle phosphorylase deficiency. Characterized by painful cramps following sustained exercise.
The rate dynamics in chemical or physical systems.
This is the active form of VITAMIN B 6 serving as a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid. During transamination of amino acids, pyridoxal phosphate is transiently converted into pyridoxamine phosphate (PYRIDOXAMINE).
The release of GLUCOSE from GLYCOGEN by GLYCOGEN PHOSPHORYLASE (phosphorolysis). The released glucose-1-phosphate is then converted to GLUCOSE-6-PHOSPHATE by PHOSPHOGLUCOMUTASE before entering GLYCOLYSIS. Glycogenolysis is stimulated by GLUCAGON or EPINEPHRINE via the activation of PHOSPHORYLASE KINASE.
An enzyme of the transferase class that catalyzes the reaction RNA(n+1) and orthophosphate to yield RNA(n) and a nucleoside diphosphate, or the reverse reaction. ADP, IDP, GDP, UDP, and CDP can act as donors in the latter case. (From Dorland, 27th ed) EC 2.7.7.8.
Conversion of an inactive form of an enzyme to one possessing metabolic activity. It includes 1, activation by ions (activators); 2, activation by cofactors (coenzymes); and 3, conversion of an enzyme precursor (proenzyme or zymogen) to an active enzyme.
Unstriated and unstriped muscle, one of the muscles of the internal organs, blood vessels, hair follicles, etc. Contractile elements are elongated, usually spindle-shaped cells with centrally located nuclei. Smooth muscle fibers are bound together into sheets or bundles by reticular fibers and frequently elastic nets are also abundant. (From Stedman, 25th ed)
A process leading to shortening and/or development of tension in muscle tissue. Muscle contraction occurs by a sliding filament mechanism whereby actin filaments slide inward among the myosin filaments.
Pentosyltransferases that catalyze the reaction between a pyrimidine nucleoside and orthophosphate to form a free pyrimidine and ribose-5-phosphate.
Large, multinucleate single cells, either cylindrical or prismatic in shape, that form the basic unit of SKELETAL MUSCLE. They consist of MYOFIBRILS enclosed within and attached to the SARCOLEMMA. They are derived from the fusion of skeletal myoblasts (MYOBLASTS, SKELETAL) into a syncytium, followed by differentiation.
The nonstriated involuntary muscle tissue of blood vessels.
1,4-alpha-D-Glucan-1,4-alpha-D-glucan 4-alpha-D-glucosyltransferase/dextrin 6 alpha-D-glucanohydrolase. An enzyme system having both 4-alpha-glucanotransferase (EC 2.4.1.25) and amylo-1,6-glucosidase (EC 3.2.1.33) activities. As a transferase it transfers a segment of a 1,4-alpha-D-glucan to a new 4-position in an acceptor, which may be glucose or another 1,4-alpha-D-glucan. As a glucosidase it catalyzes the endohydrolysis of 1,6-alpha-D-glucoside linkages at points of branching in chains of 1,4-linked alpha-D-glucose residues. Amylo-1,6-glucosidase activity is deficient in glycogen storage disease type III.
Polyhydric alcohols having no more than one hydroxy group attached to each carbon atom. They are formed by the reduction of the carbonyl group of a sugar to a hydroxyl group.(From Dorland, 28th ed)
Developmental events leading to the formation of adult muscular system, which includes differentiation of the various types of muscle cell precursors, migration of myoblasts, activation of myogenesis and development of muscle anchorage.
A class of protein-serine-threonine kinases that was originally found as one of the three types of kinases that phosphorylate GLYCOGEN SYNTHASE. Glycogen synthase kinases along with CA(2+)-CALMODULIN DEPENDENT PROTEIN KINASES and CYCLIC AMP-DEPENDENT PROTEIN KINASES regulate glycogen synthase activity.
A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal GLUCAGON-LIKE PEPTIDES. Glucagon is secreted by PANCREATIC ALPHA CELLS and plays an important role in regulation of BLOOD GLUCOSE concentration, ketone metabolism, and several other biochemical and physiological processes. (From Gilman et al., Goodman and Gilman's The Pharmacological Basis of Therapeutics, 9th ed, p1511)
A genus of velvety smooth SPONGES in the family Suberitidae, characterized by the ectosomal and choanosomal skeletons dominated by tylostyles (pin-like spicules with a pinched bulbous end).
A group of enzymes removing the SERINE- or THREONINE-bound phosphate groups from a wide range of phosphoproteins, including a number of enzymes which have been phosphorylated under the action of a kinase. (Enzyme Nomenclature, 1992)
Enzymes of the transferase class that catalyze the transfer of a pentose group from one compound to another.
A normal intermediate in the fermentation (oxidation, metabolism) of sugar. The concentrated form is used internally to prevent gastrointestinal fermentation. (From Stedman, 26th ed)
A eukayrotic protein serine-threonine phosphatase subtype that dephosphorylates a wide variety of cellular proteins. The enzyme is comprised of a catalytic subunit and regulatory subunit. Several isoforms of the protein phosphatase catalytic subunit exist due to the presence of multiple genes and the alternative splicing of their mRNAs. A large number of proteins have been shown to act as regulatory subunits for this enzyme. Many of the regulatory subunits have additional cellular functions.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
An acetic acid ester of CARNITINE that facilitates movement of ACETYL COA into the matrices of mammalian MITOCHONDRIA during the oxidation of FATTY ACIDS.
A site on an enzyme which upon binding of a modulator, causes the enzyme to undergo a conformational change that may alter its catalytic or binding properties.
An enzyme of the PHOSPHORYLASES family that catalyzes the degradation of starch, a mixture of unbranched AMYLOSE and branched AMYLOPECTIN compounds. This phosphorylase from plants is the counterpart of GLYCOGEN PHOSPHORYLASE in animals that catalyzes the reaction of inorganic phosphate on the terminal alpha-1,4-glycosidic bond at the non-reducing end of glucans resulting in the release of glucose-1-phosphate.
Genetically identical individuals developed from brother and sister matings which have been carried out for twenty or more generations or by parent x offspring matings carried out with certain restrictions. This also includes animals with a long history of closed colony breeding.
A metabolic process that converts GLUCOSE into two molecules of PYRUVIC ACID through a series of enzymatic reactions. Energy generated by this process is conserved in two molecules of ATP. Glycolysis is the universal catabolic pathway for glucose, free glucose, or glucose derived from complex CARBOHYDRATES, such as GLYCOGEN and STARCH.
An adenine nucleotide containing one phosphate group which is esterified to both the 3'- and 5'-positions of the sugar moiety. It is a second messenger and a key intracellular regulator, functioning as a mediator of activity for a number of hormones, including epinephrine, glucagon, and ACTH.
The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.
A methylxanthine naturally occurring in some beverages and also used as a pharmacological agent. Caffeine's most notable pharmacological effect is as a central nervous system stimulant, increasing alertness and producing agitation. It also relaxes SMOOTH MUSCLE, stimulates CARDIAC MUSCLE, stimulates DIURESIS, and appears to be useful in the treatment of some types of headache. Several cellular actions of caffeine have been observed, but it is not entirely clear how each contributes to its pharmacological profile. Among the most important are inhibition of cyclic nucleotide PHOSPHODIESTERASES, antagonism of ADENOSINE RECEPTORS, and modulation of intracellular calcium handling.
The active sympathomimetic hormone from the ADRENAL MEDULLA. It stimulates both the alpha- and beta- adrenergic systems, causes systemic VASOCONSTRICTION and gastrointestinal relaxation, stimulates the HEART, and dilates BRONCHI and cerebral vessels. It is used in ASTHMA and CARDIAC FAILURE and to delay absorption of local ANESTHETICS.
The modification of the reactivity of ENZYMES by the binding of effectors to sites (ALLOSTERIC SITES) on the enzymes other than the substrate BINDING SITES.
Benzopyrroles with the nitrogen at the number one carbon adjacent to the benzyl portion, in contrast to ISOINDOLES which have the nitrogen away from the six-membered ring.
Organic compounds containing the -CO-NH2 radical. Amides are derived from acids by replacement of -OH by -NH2 or from ammonia by the replacement of H by an acyl group. (From Grant & Hackh's Chemical Dictionary, 5th ed)
A strain of albino rat developed at the Wistar Institute that has spread widely at other institutions. This has markedly diluted the original strain.
Biosynthesis of GLUCOSE from nonhexose or non-carbohydrate precursors, such as LACTATE; PYRUVATE; ALANINE; and GLYCEROL.
Compounds or agents that combine with an enzyme in such a manner as to prevent the normal substrate-enzyme combination and the catalytic reaction.
A state arrived at through prolonged and strong contraction of a muscle. Studies in athletes during prolonged submaximal exercise have shown that muscle fatigue increases in almost direct proportion to the rate of muscle glycogen depletion. Muscle fatigue in short-term maximal exercise is associated with oxygen lack and an increased level of blood and muscle lactic acid, and an accompanying increase in hydrogen-ion concentration in the exercised muscle.
The introduction of a phosphoryl group into a compound through the formation of an ester bond between the compound and a phosphorus moiety.
A plant genus of the family LINACEAE that is cultivated for its fiber (manufactured into linen cloth). It contains a trypsin inhibitor and the seed is the source of LINSEED OIL.
A 51-amino acid pancreatic hormone that plays a major role in the regulation of glucose metabolism, directly by suppressing endogenous glucose production (GLYCOGENOLYSIS; GLUCONEOGENESIS) and indirectly by suppressing GLUCAGON secretion and LIPOLYSIS. Native insulin is a globular protein comprised of a zinc-coordinated hexamer. Each insulin monomer containing two chains, A (21 residues) and B (30 residues), linked by two disulfide bonds. Insulin is used as a drug to control insulin-dependent diabetes mellitus (DIABETES MELLITUS, TYPE 1).
Skeletal muscle fibers characterized by their expression of the Type II MYOSIN HEAVY CHAIN isoforms which have high ATPase activity and effect several other functional properties - shortening velocity, power output, rate of tension redevelopment. Several fast types have been identified.
Inorganic salts of phosphoric acid.
A basic element found in nearly all organized tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes.
An inorganic compound that is used as a source of iodine in thyrotoxic crisis and in the preparation of thyrotoxic patients for thyroidectomy. (From Dorland, 27th ed)
The resection or removal of the innervation of a muscle or muscle tissue.
The 4-carboxyaldehyde form of VITAMIN B 6 which is converted to PYRIDOXAL PHOSPHATE which is a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid.
The parts of a macromolecule that directly participate in its specific combination with another molecule.
Skeletal muscle fibers characterized by their expression of the Type I MYOSIN HEAVY CHAIN isoforms which have low ATPase activity and effect several other functional properties - shortening velocity, power output, rate of tension redevelopment.
Salts or esters of LACTIC ACID containing the general formula CH3CHOHCOOR.
Lengthy and continuous deprivation of food. (Stedman, 25th ed)
An adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.
The facilitation of a chemical reaction by material (catalyst) that is not consumed by the reaction.
Mitochondria of skeletal and smooth muscle. It does not include myocardial mitochondria for which MITOCHONDRIA, HEART is available.
Structurally related forms of an enzyme. Each isoenzyme has the same mechanism and classification, but differs in its chemical, physical, or immunological characteristics.
The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.
An inhibitor of ALPHA-GLUCOSIDASES that retards the digestion and absorption of DIETARY CARBOHYDRATES in the SMALL INTESTINE.
A family of enzymes that catalyze the conversion of ATP and a protein to ADP and a phosphoprotein.
Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.
The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.
An anabolic steroid used mainly as an anabolic agent in veterinary practice.
Non-striated, elongated, spindle-shaped cells found lining the digestive tract, uterus, and blood vessels. They are derived from specialized myoblasts (MYOBLASTS, SMOOTH MUSCLE).
RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.
A division of organisms that exist vegetatively as complex mobile plasmodia, reproduce by means of spores, and have complex life cycles. They are now classed as protozoa but formerly were considered fungi.
The characteristic 3-dimensional shape of a protein, including the secondary, supersecondary (motifs), tertiary (domains) and quaternary structure of the peptide chain. PROTEIN STRUCTURE, QUATERNARY describes the conformation assumed by multimeric proteins (aggregates of more than one polypeptide chain).
The sum of the weight of all the atoms in a molecule.
Antidiuretic hormones released by the NEUROHYPOPHYSIS of all vertebrates (structure varies with species) to regulate water balance and OSMOLARITY. In general, vasopressin is a nonapeptide consisting of a six-amino-acid ring with a cysteine 1 to cysteine 6 disulfide bridge or an octapeptide containing a CYSTINE. All mammals have arginine vasopressin except the pig with a lysine at position 8. Vasopressin, a vasoconstrictor, acts on the KIDNEY COLLECTING DUCTS to increase water reabsorption, increase blood volume and blood pressure.
A characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts.
Glucose in blood.
A plant genus of the family LAMIACEAE used to flavor food.
The relationship between the dose of an administered drug and the response of the organism to the drug.
The 4-methanol form of VITAMIN B 6 which is converted to PYRIDOXAL PHOSPHATE which is a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid. Although pyridoxine and Vitamin B 6 are still frequently used as synonyms, especially by medical researchers, this practice is erroneous and sometimes misleading (EE Snell; Ann NY Acad Sci, vol 585 pg 1, 1990).
Models used experimentally or theoretically to study molecular shape, electronic properties, or interactions; includes analogous molecules, computer-generated graphics, and mechanical structures.
The normality of a solution with respect to HYDROGEN ions; H+. It is related to acidity measurements in most cases by pH = log 1/2[1/(H+)], where (H+) is the hydrogen ion concentration in gram equivalents per liter of solution. (McGraw-Hill Dictionary of Scientific and Technical Terms, 6th ed)
The neck muscles consist of the platysma, splenius cervicis, sternocleidomastoid(eus), longus colli, the anterior, medius, and posterior scalenes, digastric(us), stylohyoid(eus), mylohyoid(eus), geniohyoid(eus), sternohyoid(eus), omohyoid(eus), sternothyroid(eus), and thyrohyoid(eus).
The muscles that move the eye. Included in this group are the medial rectus, lateral rectus, superior rectus, inferior rectus, inferior oblique, superior oblique, musculus orbitalis, and levator palpebrae superioris.
Inorganic compounds that contain fluorine as an integral part of the molecule.
Voluntary activity without external compulsion.
An enzyme that catalyzes the conversion of D-glucose 6-phosphate and water to D-glucose and orthophosphate. EC 3.1.3.9.
The insertion of recombinant DNA molecules from prokaryotic and/or eukaryotic sources into a replicating vehicle, such as a plasmid or virus vector, and the introduction of the resultant hybrid molecules into recipient cells without altering the viability of those cells.
Elements of limited time intervals, contributing to particular results or situations.
One of two types of muscle in the body, characterized by the array of bands observed under microscope. Striated muscles can be divided into two subtypes: the CARDIAC MUSCLE and the SKELETAL MUSCLE.
Skeletal muscle structures that function as the MECHANORECEPTORS responsible for the stretch or myotactic reflex (REFLEX, STRETCH). They are composed of a bundle of encapsulated SKELETAL MUSCLE FIBERS, i.e., the intrafusal fibers (nuclear bag 1 fibers, nuclear bag 2 fibers, and nuclear chain fibers) innervated by SENSORY NEURONS.
An enzyme that catalyzes the conversion of phosphorylated, inactive glycogen synthase D to active dephosphoglycogen synthase I. EC 3.1.3.42.
Spectroscopic method of measuring the magnetic moment of elementary particles such as atomic nuclei, protons or electrons. It is employed in clinical applications such as NMR Tomography (MAGNETIC RESONANCE IMAGING).
Inorganic chemicals that contain manganese as an integral part of the molecule.
Nucleotides in which the purine or pyrimidine base is combined with ribose. (Dorland, 28th ed)
That phase of a muscle twitch during which a muscle returns to a resting position.
The chemical or biochemical addition of carbohydrate or glycosyl groups to other chemicals, especially peptides or proteins. Glycosyl transferases are used in this biochemical reaction.
An enzyme that catalyzes the conversion of ATP and a D-hexose to ADP and a D-hexose 6-phosphate. D-Glucose, D-mannose, D-fructose, sorbitol, and D-glucosamine can act as acceptors; ITP and dATP can act as donors. The liver isoenzyme has sometimes been called glucokinase. (From Enzyme Nomenclature, 1992) EC 2.7.1.1.
An allosteric enzyme that regulates glycolysis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-1,6-bisphosphate. D-tagatose- 6-phosphate and sedoheptulose-7-phosphate also are acceptors. UTP, CTP, and ITP also are donors. In human phosphofructokinase-1, three types of subunits have been identified. They are PHOSPHOFRUCTOKINASE-1, MUSCLE TYPE; PHOSPHOFRUCTOKINASE-1, LIVER TYPE; and PHOSPHOFRUCTOKINASE-1, TYPE C; found in platelets, brain, and other tissues.
Adenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.
These include the muscles of the DIAPHRAGM and the INTERCOSTAL MUSCLES.
An autosomal recessive disease in which gene expression of glucose-6-phosphatase is absent, resulting in hypoglycemia due to lack of glucose production. Accumulation of glycogen in liver and kidney leads to organomegaly, particularly massive hepatomegaly. Increased concentrations of lactic acid and hyperlipidemia appear in the plasma. Clinical gout often appears in early childhood.
A vague complaint of debility, fatigue, or exhaustion attributable to weakness of various muscles. The weakness can be characterized as subacute or chronic, often progressive, and is a manifestation of many muscle and neuromuscular diseases. (From Wyngaarden et al., Cecil Textbook of Medicine, 19th ed, p2251)
Conical muscular projections from the walls of the cardiac ventricles, attached to the cusps of the atrioventricular valves by the chordae tendineae.
Compounds and molecular complexes that consist of very large numbers of atoms and are generally over 500 kDa in size. In biological systems macromolecular substances usually can be visualized using ELECTRON MICROSCOPY and are distinguished from ORGANELLES by the lack of a membrane structure.
The chemical reactions involved in the production and utilization of various forms of energy in cells.
Derived proteins or mixtures of cleavage products produced by the partial hydrolysis of a native protein either by an acid or by an enzyme. Peptones are readily soluble in water, and are not precipitable by heat, by alkalis, or by saturation with ammonium sulfate. (Dorland, 28th ed)
The muscle tissue of the HEART. It is composed of striated, involuntary muscle cells (MYOCYTES, CARDIAC) connected to form the contractile pump to generate blood flow.
A multienzyme complex responsible for the formation of ACETYL COENZYME A from pyruvate. The enzyme components are PYRUVATE DEHYDROGENASE (LIPOAMIDE); dihydrolipoamide acetyltransferase; and LIPOAMIDE DEHYDROGENASE. Pyruvate dehydrogenase complex is subject to three types of control: inhibited by acetyl-CoA and NADH; influenced by the energy state of the cell; and inhibited when a specific serine residue in the pyruvate decarboxylase is phosphorylated by ATP. PYRUVATE DEHYDROGENASE (LIPOAMIDE)-PHOSPHATASE catalyzes reactivation of the complex. (From Concise Encyclopedia Biochemistry and Molecular Biology, 3rd ed)
The quadriceps femoris. A collective name of the four-headed skeletal muscle of the thigh, comprised of the rectus femoris, vastus intermedius, vastus lateralis, and vastus medialis.
The study of crystal structure using X-RAY DIFFRACTION techniques. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
Treatment process involving the injection of fluid into an organ or tissue.
Proteins prepared by recombinant DNA technology.
ATP:pyruvate 2-O-phosphotransferase. A phosphotransferase that catalyzes reversibly the phosphorylation of pyruvate to phosphoenolpyruvate in the presence of ATP. It has four isozymes (L, R, M1, and M2). Deficiency of the enzyme results in hemolytic anemia. EC 2.7.1.40.
Abstaining from all food.
Substances which lower blood glucose levels.
Mature contractile cells, commonly known as myocytes, that form one of three kinds of muscle. The three types of muscle cells are skeletal (MUSCLE FIBERS, SKELETAL), cardiac (MYOCYTES, CARDIAC), and smooth (MYOCYTES, SMOOTH MUSCLE). They are derived from embryonic (precursor) muscle cells called MYOBLASTS.
Cellular processes in biosynthesis (anabolism) and degradation (catabolism) of CARBOHYDRATES.
A basic science concerned with the composition, structure, and properties of matter; and the reactions that occur between substances and the associated energy exchange.
Muscles forming the ABDOMINAL WALL including RECTUS ABDOMINIS, external and internal oblique muscles, transversus abdominis, and quadratus abdominis. (from Stedman, 25th ed)
The composition, conformation, and properties of atoms and molecules, and their reaction and interaction processes.
Purines with a RIBOSE attached that can be phosphorylated to PURINE NUCLEOTIDES.
A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.
A strain of albino rat used widely for experimental purposes because of its calmness and ease of handling. It was developed by the Sprague-Dawley Animal Company.
A purine nucleoside that has hypoxanthine linked by the N9 nitrogen to the C1 carbon of ribose. It is an intermediate in the degradation of purines and purine nucleosides to uric acid and in pathways of purine salvage. It also occurs in the anticodon of certain transfer RNA molecules. (Dorland, 28th ed)
A masticatory muscle whose action is closing the jaws.
Nucleosides in which the base moiety is substituted with one or more sulfur atoms.
An intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed)
A paravertebral sympathetic ganglion formed by the fusion of the inferior cervical and first thoracic ganglia.
The main structural component of the LIVER. They are specialized EPITHELIAL CELLS that are organized into interconnected plates called lobules.
Ribose substituted in the 1-, 3-, or 5-position by a phosphoric acid moiety.
Muscles of facial expression or mimetic muscles that include the numerous muscles supplied by the facial nerve that are attached to and move the skin of the face. (From Stedman, 25th ed)
The scattering of x-rays by matter, especially crystals, with accompanying variation in intensity due to interference effects. Analysis of the crystal structure of materials is performed by passing x-rays through them and registering the diffraction image of the rays (CRYSTALLOGRAPHY, X-RAY). (From McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
An autosomal recessively inherited glycogen storage disease caused by GLUCAN 1,4-ALPHA-GLUCOSIDASE deficiency. Large amounts of GLYCOGEN accumulate in the LYSOSOMES of skeletal muscle (MUSCLE, SKELETAL); HEART; LIVER; SPINAL CORD; and BRAIN. Three forms have been described: infantile, childhood, and adult. The infantile form is fatal in infancy and presents with hypotonia and a hypertrophic cardiomyopathy (CARDIOMYOPATHY, HYPERTROPHIC). The childhood form usually presents in the second year of life with proximal weakness and respiratory symptoms. The adult form consists of a slowly progressive proximal myopathy. (From Muscle Nerve 1995;3:S61-9; Menkes, Textbook of Child Neurology, 5th ed, pp73-4)
Muscles arising in the zygomatic arch that close the jaw. Their nerve supply is masseteric from the mandibular division of the trigeminal nerve. (From Stedman, 25th ed)
Inorganic salts of sulfuric acid.
Respiratory muscles that arise from the lower border of one rib and insert into the upper border of the adjoining rib, and contract during inspiration or respiration. (From Stedman, 25th ed)
An alpha-1 adrenergic agonist used as a mydriatic, nasal decongestant, and cardiotonic agent.
The domestic dog, Canis familiaris, comprising about 400 breeds, of the carnivore family CANIDAE. They are worldwide in distribution and live in association with people. (Walker's Mammals of the World, 5th ed, p1065)
The relationship between the chemical structure of a compound and its biological or pharmacological activity. Compounds are often classed together because they have structural characteristics in common including shape, size, stereochemical arrangement, and distribution of functional groups.
Derangement in size and number of muscle fibers occurring with aging, reduction in blood supply, or following immobilization, prolonged weightlessness, malnutrition, and particularly in denervation.
Recording of the changes in electric potential of muscle by means of surface or needle electrodes.
Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.
Chemical substances having a specific regulatory effect on the activity of a certain organ or organs. The term was originally applied to substances secreted by various ENDOCRINE GLANDS and transported in the bloodstream to the target organs. It is sometimes extended to include those substances that are not produced by the endocrine glands but that have similar effects.
Drugs that inhibit the actions of the sympathetic nervous system by any mechanism. The most common of these are the ADRENERGIC ANTAGONISTS and drugs that deplete norepinephrine or reduce the release of transmitters from adrenergic postganglionic terminals (see ADRENERGIC AGENTS). Drugs that act in the central nervous system to reduce sympathetic activity (e.g., centrally acting alpha-2 adrenergic agonists, see ADRENERGIC ALPHA-AGONISTS) are included here.
Muscular contractions characterized by increase in tension without change in length.
Isopropyl analog of EPINEPHRINE; beta-sympathomimetic that acts on the heart, bronchi, skeletal muscle, alimentary tract, etc. It is used mainly as bronchodilator and heart stimulant.
The concentration of osmotically active particles in solution expressed in terms of osmoles of solute per liter of solution. Osmolality is expressed in terms of osmoles of solute per kilogram of solvent.
Abnormally high BLOOD GLUCOSE level.
Elongated, spindle-shaped, quiescent myoblasts lying in close contact with adult skeletal muscle. They are thought to play a role in muscle repair and regeneration.
A fatty acid that is found in plants and involved in the formation of prostaglandins.
A network of tubules and sacs in the cytoplasm of SKELETAL MUSCLE FIBERS that assist with muscle contraction and relaxation by releasing and storing calcium ions.
The pectoralis major and pectoralis minor muscles that make up the upper and fore part of the chest in front of the AXILLA.
A specific inhibitor of phosphoserine/threonine protein phosphatase 1 and 2a. It is also a potent tumor promoter. (Thromb Res 1992;67(4):345-54 & Cancer Res 1993;53(2):239-41)
The region of an enzyme that interacts with its substrate to cause the enzymatic reaction.
The degree of similarity between sequences of amino acids. This information is useful for the analyzing genetic relatedness of proteins and species.
Small containers or pellets of a solid drug implanted in the body to achieve sustained release of the drug.
Acquired, familial, and congenital disorders of SKELETAL MUSCLE and SMOOTH MUSCLE.
In glycogen or amylopectin synthesis, the enzyme that catalyzes the transfer of a segment of a 1,4-alpha-glucan chain to a primary hydroxy group in a similar glucan chain. EC 2.4.1.18.
Purine bases found in body tissues and fluids and in some plants.
Determination of the spectra of ultraviolet absorption by specific molecules in gases or liquids, for example Cl2, SO2, NO2, CS2, ozone, mercury vapor, and various unsaturated compounds. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
An autosomal recessive metabolic disorder due to deficient expression of amylo-1,6-glucosidase (one part of the glycogen debranching enzyme system). The clinical course of the disease is similar to that of glycogen storage disease type I, but milder. Massive hepatomegaly, which is present in young children, diminishes and occasionally disappears with age. Levels of glycogen with short outer branches are elevated in muscle, liver, and erythrocytes. Six subgroups have been identified, with subgroups Type IIIa and Type IIIb being the most prevalent.
Expenditure of energy during PHYSICAL ACTIVITY. Intensity of exertion may be measured by rate of OXYGEN CONSUMPTION; HEAT produced, or HEART RATE. Perceived exertion, a psychological measure of exertion, is included.
The intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GAMMA-AMINOBUTYRIC ACID-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptor-mediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway.
The location of the atoms, groups or ions relative to one another in a molecule, as well as the number, type and location of covalent bonds.
Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.
The long cylindrical contractile organelles of STRIATED MUSCLE cells composed of ACTIN FILAMENTS; MYOSIN filaments; and other proteins organized in arrays of repeating units called SARCOMERES .
Use of electric potential or currents to elicit biological responses.
The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.
Either of two extremities of four-footed non-primate land animals. It usually consists of a FEMUR; TIBIA; and FIBULA; tarsals; METATARSALS; and TOES. (From Storer et al., General Zoology, 6th ed, p73)
An antineoplastic antimetabolite that is metabolized to fluorouracil when administered by rapid injection; when administered by slow, continuous, intra-arterial infusion, it is converted to floxuridine monophosphate. It has been used to treat hepatic metastases of gastrointestinal adenocarcinomas and for palliation in malignant neoplasms of the liver and gastrointestinal tract.
Detection of RNA that has been electrophoretically separated and immobilized by blotting on nitrocellulose or other type of paper or nylon membrane followed by hybridization with labeled NUCLEIC ACID PROBES.
Phosphorylase. The crystal structure of the rabbit muscle glycogen phosphorylase-AMP complex. AMP allosteric site (yellow), ... AMP activates glycogen phosphorylase b by changing its conformation from a tense to a relaxed form. This relaxed form has ... Glycogen phosphorylase is one of the phosphorylase enzymes (EC 2.4.1.1). Glycogen phosphorylase catalyzes the rate-limiting ... Mutations in the muscle isoform of glycogen phosphorylase (PYGM) are associated with glycogen storage disease type V (GSD V, ...
glycogen metabolic process. • metabolism. • lysosome organization. • striated muscle contraction. • glucose metabolic process. ... Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of ... Glycogen synthase. *Glycogen branching enzyme. *Glycogenin. Glycogenolysis. extralysosomal:. *Glycogen phosphorylase. * ... cardiac muscle contraction. • muscle cell cellular homeostasis. • neuromuscular process controlling posture. • locomotory ...
... stores in skeletal muscle serve as a form of energy storage for the muscle itself; however, the breakdown of muscle ... Due to the way glycogen is synthesised, every glycogen granule has at its core a glycogenin protein. Glycogen is in muscle, ... Glycogen is cleaved from the nonreducing ends of the chain by the enzyme glycogen phosphorylase to produce monomers of glucose- ... In skeletal muscle, glycogen is found in a low concentration (1-2% of the muscle mass) and the skeletal muscle of an adult ...
In addition to glycogen breakdown with the glycogen debranching enzyme and the glycogen phosphorylase enzyme, cells also use ... is an energy storage form for carbohydrates in many human cells; this is most important in liver, muscle and certain brain ... G-1-P is converted into UDP-glucose by the action of the enzyme UDP-glucose pyrophosphorylase (UGP). The enzyme glycogenin (GYG ... Glycogen storage diseases are enzyme deficiencies which impair glycogen synthesis, glycogen degradation or glycolysis. The two ...
... whereas muscle glycogen synthesis accounts for disposal of up to 90% of ingested glucose. The role of muscle glycogen is as a ... Since the structure of eukaryotic glycogen synthase is highly conserved among species, glycogen synthase likely forms a ... Much research has been done on glycogen degradation through studying the structure and function of glycogen phosphorylase, the ... However, since glycogen synthase requires an oligosaccharide primer as a glucose acceptor, it relies on glycogenin to initiate ...
These include glycogen synthase, phosphorylase, and branching and debranching enzymes for glycogen. All of these are regulatory ... The proteins are the enzymes that are associated with the metabolism of glycogen. These proteins and glycogen form a complex to ... The initiation of synthesis of glycogen requires glycogenin, found in glycosomes, a protein primer. Glycogen synthase as ... This accounts for why desmoglycosomes are found in muscle cells. These glycosomes are not affected by acid. These glycosomes ...
... glycogen synthase (GS), and degradation, glycogen phosphorylase (GF). Glycogenin is the initiator of the glycogen biosynthesis ... cardiac muscle and other types of tissue, but not in skeletal muscle. Glycogenin-1 was described by analyzing the glycogen of ... Glycogenin-1 is an enzyme that is involved in the biosynthesis of glycogen. It is capable of self-glucosylation, forming an ... glycogen is used as an energy source for muscle contraction during exercise. The different functions of glycogen in muscle or ...
102513604 PYGM; glycogen phosphorylase, muscle associated 102513352 PYGB; glycogen phosphorylase, brain form 102515206 AGL; ... 102511249 GYS1; glycogen synthase 1 102511313 GYS2; glycogen synthase 2 102509684 GYG1; glycogenin 1 102514856 GYG2; glycogenin ... glycogen phosphorylase L 102513604 PYGM; glycogen phosphorylase, muscle associated 102513352 PYGB; glycogen phosphorylase, ... glycogen phosphorylase L 102513604 PYGM; glycogen phosphorylase, muscle associated 102513352 PYGB; glycogen phosphorylase, ...
... glycogen synthase (GS), and degradation, glycogen phosphorylase (GF). Glycogenin is the initiator of the glycogen biosynthesis ... cardiac muscle and other types of tissue, but not in skeletal muscle. Glycogenin-1 was described by analyzing the glycogen of ... Glycogenin-1 is an enzyme that is involved in the biosynthesis of glycogen. It is capable of self-glucosylation, forming an ... glycogen is used as an energy source for muscle contraction during exercise. The different functions of glycogen in muscle or ...
However, glycogen is different because it is a storage form of energy. Glycogen is normally stored in either the muscles or the ... there is a protein by the name of glycogenin which acts like a primer and initially starts the reaction to form glycogen. ... Similar to the glycogen synthase, the phosphorylase cannot remove glucose 4 residues from the end of one chain or branch. There ... Therefore, the glycogen stored in muscle is meant to be used for the muscle, and also helps when muscles are in need of glucose ...
... whereas muscle glycogen synthesis accounts for disposal of up to 90% of ingested glucose. The role of muscle glycogen is as a ... Since the structure of eukaryotic glycogen synthase is highly conserved among species, glycogen synthase likely forms a ... Much research has been done on glycogen degradation through studying the structure and function of glycogen phosphorylase, the ... However, since glycogen synthase requires an oligosaccharide primer as a glucose acceptor, it relies on glycogenin to initiate ...
... actives phosphorylase kinase These will ... - A free PowerPoint PPT presentation (displayed as a Flash slide show) on PowerShow ... Glycogen Glycogen Synthesis Glycogen Synthase 1-4 bonds Summary Skeletal Muscle Epi/N-Epi (beta receptor) C-AMP Ca++ ( ... Size glycogenin Proglycogen Macroglycogen 12 tiers is the maximum branches too dense to allow GS 7. Two Forms of Muscle ... Glycogen. Description:. Glycogen Glycogen Synthesis Glycogen Synthase 1-4 bonds Summary Skeletal Muscle Epi/N-Epi (beta ...
Glycogenin-linked maltooctasaccharides thus formed are used as primers by GS, and apparently glycogenin remains linked to the ... They code for glycogen phosphorylase, glycogen-branching enzyme, ADP-Glc pyrophosphorylase, GS, phosphoglucomutase, and ... A somewhat similar approach has been used to synthesize Glc-free recombinant muscle glycogenin in E. coli. In this case, ... All genes involved in known Agrobacterium tumefaciens glycogen metabolism steps are clustered in a single operon formed by the ...
... metabolic disorders that result from a defect in any one of several enzymes required for either glycogen synthesis or glycogen ... The glycogen storage diseases (GSDs) are a group of inherited ... Phosphorylase kinase, α subunit (muscle). PHKA1. Xq13. X-linked ... Glycogen storage disease type V (OMIM 232600) is a pure myopathic form of GSD affecting skeletal muscle. This disease was the ... or can be added to the protein glycogenin to create a new glycogen molecule. Glycogen synthase catalyzes the formation of α-1,4 ...
Glycogen is a strongly branched polymer of α-D-glucose, with glucose residues in the linear chains linked by 1→4-bonds (~93% of ... 8. Structures of the muscle-phospho-rylase limit dextrins of glycogen and amylopectin, Biochem. J., 76, 264-270.PubMed ... It is thought currently that a fully formed glycogen molecule (β-particle) with the self-glycosylating protein glycogenin in ... glycogen molecule α-particles β-particles polysaccharide-protein complex glycogen fractions liver skeletal muscles ...
AMP activates glycogen phosphorylase b by changing its conformation from a tense to a relaxed form. This relaxed form has ... In humans, there are two isoforms of glycogenin - glycogenin-1, encoded by GYG1, and expressed in muscle; and glycogenin-2, ... Glycogen phosphorylase[edit]. Glycogen phosphorylase is one of the phosphorylase enzymes (EC 2.4.1.1). Glycogen phosphorylase ... Glycogen phosphorylase kinase activates glycogen phosphorylase in the same manner mentioned previously. Glycogen phosphorylase ...
In contrast, in muscle, these processes are regulated to meet the energy needs of the muscle itself. Glycogen synthesis is, ... Phosphorylase Kinase. Ser/Thr Protein Phosphatase 2A. Glycogen (n+1). Glucose 1-phosphate. Ppp2r2b. Gyg. Glucose 6-phosphate. ... the protein glycogenin is needed to initiate the synthesis of glycogen. The glycogen-branching enzyme, amylo (a1-,4) to (a1-,6 ... Energy for glycogen synthesis comes from UTP, which reacts with glucose-1-phosphate, forming UDP-glucose, in reaction catalyzed ...
... the primary carbohydrate stored in the liver and muscle cells of animals, is broken down into glucose to ... One of the main forms of control is the varied phosphorylation of glycogen synthase and glycogen phosphorylase by protein ... Schematic two-dimensional cross-sectional view of glycogen: A core protein of glycogenin is surrounded by branches of glucose ... Activated PP1 directly glycogenwsis glycogen phosphorylase a, reforming the inactive glycogen phosphorylase b, whereas ...
... whereas muscle glycogen synthesis accounts for disposal of up to 90% of ingested glucose. The role of muscle glycogen is as a ... Since the structure of [[eukaryotic]] glycogen synthase is highly conserved among species, glycogen synthase likely forms a ... Much research has been done on glycogen degradation through studying the structure and function of glycogen phosphorylase, the ... glycogenin]] to initiate de novo glycogen synthesis.,ref name=pmid23134486/> In a recent study of [[genetically modified ...
... insoluble form of glycogen. Loss-of-function mutations either in the EPM2A or in the NHLRC1 gene lead to polyglucosan formation ... an increased glycogen phosphate leads to glycogen conformational change, unfolding, precipitation, and conversion to ... One hypothesis on LB formation is based on findings that laforin-malin complex downregulates glycogen synthase (GS) through ... polyglucosan, while GS remains bound to the precipitating glycogen. In this review, we summarize all the recent findings that ...
Glycogen Synthase. Forms alfa-(1 4) glycosidic bonds in glycogen. Glycogenin (a protein!) forms the core of a. glycogen ... Muscle contains a high level of glycogen with normal structure. Type VI: Hers Disease; Liver Glycogen Phosphorylase Defect ... More muscle than liver, therefore more glycogen in muscle, overall. Use glycogen (i.e., glucose) for energy only (no export of ... Type V: McArdle Disease; Skeletal Muscle Glycogen Phosphorylase Defect. Skeletal muscle is affected, whereas the liver enzyme ...
Thus, both short and long wake lead to increased glycogen turnover around cortical synapses, whereas sleep promotes glycogen ... Since brain metabolism is higher in wake than in sleep, it was hypothesized that glycogen stores are depleted during wake and ... Since brain metabolism is higher in wake than in sleep, it was hypothesized that glycogen stores are depleted during wake and ... Relative to sleep, all wake conditions increased the number of glycogen granules around the synapses to a similar extent. ...
McArdle disease occurs due to a defect in the isoform of glycogen phosphorylase found in muscle. ... Each of these glycogen molecules form so-called β-particles. 20 - 40 β-particles form a so-called α-rosette, which can be ... Which enzymes are involved in the synthesis of glycogen? *Glycogenin, glycogen synthase ... Glycogen is degraded by glycogen phosphorylase and debranching enzyme. Glycogen phosphorylase converts the glucose units into ...
A point mutation in glycogenin causes a stop codon to form where a tyrosine residue should be. Which of the following would you ... Phosphorylation of Glycogen Synthase by Glycogen Phosphorylase. Phosphorylation of Glycogen Phosphorylase by Phosphorylase ... of the following occurs during Glycogenolysis to maintain Tammys blood glucose levels and fuel exercising skeletal muscle ... Phosphorylation of Phosphorylase Kinase by Glycogen Phosphorylase. Phosphorylation of Glycogen Synthase by Phosphorylase Kinase ...
... branched polysaccharide that is the main storage form of glucose in animals and humans. Glycogen is as an important energy ... reservoir; when energy is required by the body, glycogen in broken down to glucose, which then enters the glycolytic or pentose ... the glycogen phosphorylase isoform present in muscle. Symptoms are often observed in children, but the disease may not be ... The protein glycogenin, which is involved in glycogen synthesis, is located at the core of each glycogen granule. Glycogen is ...
1983). Comparison of A M P and N A D H binding to glycogen phosphorylase b. J Mol Biol 170(2): 529-65. Sujatha, M . S. and P ... The closest match obtained was that of rabbit muscle glycogenin (Gibbons, Roach et al. 2002) (PDB code 1112) with a r.m.s. ... oxidized form) N A D H nicotinamide adenine dinucleotide (reduced form) N C A M neural cell adhesion molecule NDP nucleoside ... 2002). Crystal structure of the autocatalytic initiator of glycogen biosynthesis, glycogenin. J Mol Biol 319(2): 463-77. 132 ...
PYGM] Glycogen phosphorylase, muscle form (EC 2.4.1.1) (Myophosphorylase). [SCGB1A1 UGB UGL] Uteroglobin (Blastokinin) ( ... GYG1 GYG] Glycogenin-1 (GN-1) (GN1) (EC 2.4.1.186). [SLC7A5] Large neutral amino acids transporter small subunit 1 (4F2 light ... ACTA1 ACTA] Actin, alpha skeletal muscle (Alpha-actin-1) [Cleaved into: Actin, alpha skeletal muscle, intermediate form]. [TNF ... soluble form]. [PKM PKM2] Pyruvate kinase PKM (EC 2.7.1.40) (Pyruvate kinase muscle isozyme). [PFKM] ATP-dependent 6- ...
PYGM] Glycogen phosphorylase, muscle form (EC 2.4.1.1) (Myophosphorylase). [SCGB1A1 UGB UGL] Uteroglobin (Blastokinin) ( ... GYG1 GYG] Glycogenin-1 (GN-1) (GN1) (EC 2.4.1.186). [SLC7A5] Large neutral amino acids transporter small subunit 1 (4F2 light ... ACTA1 ACTA] Actin, alpha skeletal muscle (Alpha-actin-1) [Cleaved into: Actin, alpha skeletal muscle, intermediate form]. [TNF ... soluble form]. [PKM PKM2] Pyruvate kinase PKM (EC 2.7.1.40) (Pyruvate kinase muscle isozyme). [PFKM] ATP-dependent 6- ...
Muscle glycogenolysis.. Glycogen breakdown is regulated by glycogen phosphorylase. It is interesting that although muscle ... Effects of exercise on GLUT-4 and glycogenin gene expression in human skeletal muscle. J Appl Physiol 88:794-796, 2000. ... phosphorylase transformation to its active phosphorylated form is not (29). This suggests that allosteric regulators may be ... and muscle glycogen. Muscle glycogen is the chief source of energy during the early stages of strenuous exercise, while with ...
Cyclic AMP elevator compositions are shown to reduce glycogen storage in affected cells and therefore can be used to treat or ... reduce symptoms in subjects having glycogen storage diseases or conditions with a buildup of glycogen. ... The invention provides compositions and methods for treating glycogen storage diseases or conditions with a buildup of glycogen ... glycogen debranching enzyme, glycogen branching enzyme, muscle glycogen phosphorylase, liver glycogen phosphorylase, muscle ...
Increased glycogen synthesis insulin forces storage of glucose in liver (and muscle) cells in the form of glycogen; lowered ... What happens then in new cells to make now glycogen granules? Can use a special primer protein (glycogenin). Thus glycogen ... This is a major advantage in anaerobic tissues, get one more ATP/glucose (3 instead of 2!). [Phosphorylase was originally ... Glycogen:. Glycogen is the storage form of glucose in animals and humans which is analogous to the starch in plants. Glycogen ...
... forms. The proportion of proglycogen to macroglycogen varies widely between liver, skeletal muscle, and heart, from 3 to 15% to ... The amount of glycogenin will influence how much glycogen the cell can store. Therefore, the production of active glycogenin ... mechanisms of protein phosphorylation/dephosphorylation that regulate the activities of glycogen synthase and phosphorylase. ... form of glycogen that serves as a stable intermediate on the pathways to and from depot glycogen (macroglycogen, mass 10(7) Da ...
Study Flashcards On glycogen metabolism at Cram.com. Quickly memorize the terms, phrases and much more. Cram.com makes it easy ... activates the enzyme which leads to the Dephosphorylation of: phosphorylase kinase, glycogen synthase, glycogen phosphorylase ... what, self-glycosylating, protein forms the initial glucose monomer chain from which glycogen can be formed? ... what two effects does insulin binding to muscle cells have on the glucose transporter GLUT-4? ...
Glycogen is an essential storage molecule for carbohydrates in the human body. It is a complex polymer consisting of multiple ... Muscle contraction: increases intracellular calcium levels → ↑ calmodulin * Activates glycogen phosphorylase kinase, which ... Glycogen synthesis 1.) Synthesis of UDP-glucose * UDP-glucose: activated form of glucose; building block for glycogen synthesis ... Glycogenin *Homodimer protein at the core of each glycogen unit. *Creates the starting point of glycogen synthesis by ...
glycogen synthesis C. glycogen synthetase forms the 1-4 glycosidic backbone true ... Lactate is the end-product of glycolysis in vigorously exercised leg white (fast twitch) muscles ... glycogen synthesisB. glycogen synthetase requires glycogenin to produce a primer true 22 of 41 ... in glycogen degredation: glycogen phosphorylase hydrolyses 1-4 glycosidic bonds true 16 of 41 ...
In muscle, the binding of an AMP molecule to glycogen phosphorylase enzyme shifts the T-state glycogen phosphorylase to R-state ... Figure 1: Overview of Glycogen synthesis (Glycogenesis). Synthesis of Glycogen. The addition of glucose to form glycogen ... During de novo synthesis, glucose molecules are added to tyrosine residues of primer protein glycogenin. The enzyme Glycogen ... In animals and humans; glucose is stored in the form of glycogen in the liver (~10%) and muscles (~2%). These stored glycogen ...
Glycogen Metabolism and Gluconeogenesis CH 339K Glycolysis (recap) We discussed the reactions which convert glucose to pyruvate ... Mature GlycogenBuilt around glycogenin coreMultiple non-reducing ends accessible to glycogen phosphorylase ... Gluconeogenesis - JU Medicine ≈ 75 g ( liver glycogen); enough for 16 hours ≈ 400 g (muscle glycogen);. Documents ... Ga contains the nucleotide binding site, holding GDP in the inactive form, and is the warhead of the G protein. At least 20 ...
  • There is another enzyme called the "debranching enzyme" which can completely degrade these parts of glycogen molecules. (wikibooks.org)
  • When glycogen phosphorylase has reached the last 4 glucose units of a branch, the transferase activity of debranching enzyme takes the outermost 3 glucose units and puts them on the "main chain", while leaving a branch of just 1 glucose. (greek.doctor)
  • Glycogen is broken down via glycogen phosphorylase and releases glucose-1-phosphate molecules -once about 4 residues are left a transferase activity of debranching enzyme takes 3 of the four glucose molecules and puts them on the end of the chain. (brainscape.com)
  • Afterwards, glycogen debranching enzyme takes over. (cram.com)
  • What are the two enzyme activities associated with glycogen debranching enzyme on the same polypeptide? (cram.com)
  • How many isoforms exist for glycogen phosphorylase and glycogen debranching enzyme? (cram.com)
  • It is functionally similar to glycogen debranching enzyme , but is on a different chromosome, processed differently by the cell and is located in the lysosome rather than the cytosol. (wikipedia.org)
  • It also plays a role in glycogen metabolism regulation. (wikipedia.org)
  • The different functions of glycogen in muscle or liver make the regulation mechanisms of its metabolism differ in each tissue. (wikipedia.org)
  • All genes involved in known Agrobacterium tumefaciens glycogen metabolism steps are clustered in a single operon formed by the sequentially transcribed glgP, glgB, glgC, glgA, pgm , and glgDB genes ( 4 , 5 ). (pnas.org)
  • The control of glycogen synthase is a key step in regulating glycogen metabolism and glucose storage. (wikipedia.org)
  • Greenberg, C. C., Jurczak, M. J., Danos, A. M., and Brady, M. J. (2006) Glycogen branches out: new perspec-tives on the role of glycogen metabolism in the integration of metabolic pathways , Am. J. Physiol. (springer.com)
  • Glycogen degradation consists of three steps: (1) the release of glucose 1-phosphate from glycogen, (2) the remodeling of the glycogen substrate to permit further degradation, and (3) the conversion of glucose 1-phosphate into glucose 6-phosphate for further metabolism. (wikipathways.org)
  • Since brain metabolism is higher in wake than in sleep, it was hypothesized that glycogen stores are depleted during wake and replenished during sleep. (frontiersin.org)
  • The basic steps in glucose metabolism are glycogenesis , or glycogen synthesis, and glycogenolysis, or glycogen breakdown. (biologydictionary.net)
  • Regulation of glycogen metabolism. (tdmuv.com)
  • The discovery of glycogenin as a self-glucosylating protein that primes glycogen synthesis has significantly increased our understanding of the structure and metabolism of this storage polysaccharide. (semanticscholar.org)
  • Glycogen metabolism is regulated mainly by hormones . (amboss.com)
  • Glycogen Metabolism and Gluconeogenesis CH 339K. (pdfslide.net)
  • Glycogen metabolism is regulated by allosteric modification and covalent modifications. (medicalbiochemist.com)
  • ATP, phosphocreatine (PCr), glycogen, and glucose concentrations were measured to investigate energy metabolism homeostasis. (diabetesjournals.org)
  • Glycogen metabolism is an intricate process involving many enzymes and cofactors resulting in the regular release and storage of glucose. (wikibooks.org)
  • The control of glycogen metabolism by the enzymes phosphorylase and glycogen synthetase is effected in animal tissues mainly through the inter conversions of the active and inactive forms of these enzymes. (thefreedictionary.com)
  • The aim of this study is to investigate the effect of 14 days of treatment with the dietary oil-supplement Triheptanoin on fat metabolism and exercise tolerance in patients with Phosphofructokinase deficiency, Debrancher deficiency and Glycogenin-1 deficiency. (clinicaltrials.gov)
  • It was determined that this molecule was united by a covalent bond to each mature molecule of muscular glycogen. (wikipedia.org)
  • In other words, glycogen is a molecule constituted of numerous glucose molecules. (wikibooks.org)
  • It is thought currently that a fully formed glycogen molecule (β-particle) with the self-glycosylating protein glycogenin in the center has a spherical shape with diameter of ~42 nm and contains ~ 55,000 glucose residues. (springer.com)
  • The glycogen molecule also includes numerous proteins involved in its synthesis and degradation, as well as proteins performing a carcass function. (springer.com)
  • This review presents the available data on the spatial structure of the glycogen molecule and its changes under various physiological and pathological conditions. (springer.com)
  • Glycogen is the molecule that functions as the secondary long-term energy storage in animal and fungal cells. (wikibooks.org)
  • 6) transglycosylase, catalyzes the transfer of a terminal fragment of 6-7 glucose residues from a nonreducing end to the C-6 hydroxyl group of a glucose residue deeper into the interior of the glycogen molecule. (wikipathways.org)
  • The significance of the multi-branched structure is that multiple glucose units, rather than a single glucose can be mobilized from any glycogen molecule when glycogenolysis is tlycogenesis. (gsdhelp.info)
  • Glycogen is the most important molecule for storing carbohydrates in humans. (greek.doctor)
  • Glycogen is a large molecule, containing up to 50 000 units of glucose. (greek.doctor)
  • Because it is branched, the glycogen molecule has many ends, however it only has one reducing end - all the other ends are non-reducing . (greek.doctor)
  • For the creation of the branches in the glycogen molecule, glycogen branching enzyme is needed. (greek.doctor)
  • Glycogen is an essential storage molecule for carbohydrates in the human body. (amboss.com)
  • what specific bond is associated with branching on a glycogen molecule? (cram.com)
  • branching on a glycogen molecule occurs at approximately every ______ glucose monomers. (cram.com)
  • a 7 segment section is transferred from the non-reducing endof a chain to the C6-hydroxyl group on the same or another glycogen molecule. (cram.com)
  • first, the enzyme transfers the last three residues on the branch chain to the non-reducing end of another glycogen molecule. (cram.com)
  • The addition of glucose to form glycogen requires a primer molecule where the glucose can be added to the non-reducing ends. (medicalbiochemist.com)
  • The enzyme Glycogen synthase catalyzes the addition of glucose molecules at the nonreducing end of core glycogen molecule In this reaction, an activated UDP-glucose molecule forms 1-4 glycosidic linkage with existing glucose moiety of glycogen molecule and free UDP is liberated. (medicalbiochemist.com)
  • This results in the elongation of a glycogen molecule with the addition of one glucose moiety in each reaction. (medicalbiochemist.com)
  • Glycogen is left with one fewer glucose molecule , and the free glucose molecule is in the form of glucose-1-phosphate . (wikipedia.org)
  • Once the Schiff base linkage is formed, holding the PLP molecule in the active site, the phosphate group on the PLP readily donates a proton to an inorganic phosphate molecule, allowing the inorganic phosphate to in turn be deprotonated by the oxygen forming the α-1,4 glycosidic linkage. (wikipedia.org)
  • The protonated oxygen now represents a good leaving group , and the glycogen chain is separated from the terminal glycogen in an S N 1 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)
  • What is the molecule called after complete digestion by glycogen phosphorylase? (cram.com)
  • These mechanisms are based mainly on the differences on structure and on the regulation of the enzymes that catalyze synthesis, glycogen synthase (GS), and degradation, glycogen phosphorylase (GF). (wikipedia.org)
  • Much research has been done on glycogen degradation through studying the structure and function of glycogen phosphorylase, the key regulatory enzyme of glycogen degradation. (wikipedia.org)
  • The glycogen storage diseases (GSDs) are a group of inherited metabolic disorders that result from a defect in any one of several enzymes required for either glycogen synthesis or glycogen degradation. (iospress.com)
  • Blood glucose can be obtained from three primary sources: diet, degradation of glycogen, and gluconeogenesis. (iospress.com)
  • In the liver, glycogen synthesis and degradation are regulated to maintain blood-glucose levels as required to meet the needs of the organism as a whole. (wikipathways.org)
  • What are the two major enzymes involved in glycogen degradation? (cram.com)
  • This metabolic process is in turn broken down to glycogen degradation and synthesis. (wikibooks.org)
  • This occurs in part as a result of poor movement of GLUT4 glucose transporters to the cell membrane, leading to insulin resistance, along with low muscle glycogen synthetase activity, leading to poor glycogen storage, in a counter-regulatory hormonal environment that enhances muscle protein degradation due to glucoeneogenesis. (thefreedictionary.com)
  • This gene encodes lysosomal alpha-glucosidase , which is essential for the degradation of glycogen to glucose in lysosomes . (wikipedia.org)
  • Glycogen synthase (UDP-glucose-glycogen glucosyltransferase) is a key enzyme in glycogenesis, the conversion of glucose into glycogen. (wikipedia.org)
  • It is made primarily by the liver and the muscles, but can also be made by glycogenesis within the brain and stomach. (wikibooks.org)
  • Glycogenolysis is the biochemical breakdown of glycogen to glucose whereas glycogenesis is the opposite, the formation of glycogen from glucose. (gsdhelp.info)
  • The reverse process, glycogenesis, the formation of glycogen from glucose, occurs in liver and muscle cells when glucose and ATP are present in relatively high amounts. (gsdhelp.info)
  • Glucose is also stored as glycogen (glycogenesis). (scribd.com)
  • The glucose that is not used immediately is converted in the liver and muscles into glycogen for storage by the process of glycogenesis. (tdmuv.com)
  • In the human body, the two main tissues which store glycogen are liver and skeletal muscle. (wikipedia.org)
  • In skeletal muscle, glycogen is used as an energy source for muscle contraction during exercise. (wikipedia.org)
  • and glycogenin-2, with a molecular weight of 66 kDa and codified by GYG2 gen, which is expressed mainly in liver, cardiac muscle and other types of tissue, but not in skeletal muscle. (wikipedia.org)
  • Glycogenin-1 was described by analyzing the glycogen of skeletal muscle. (wikipedia.org)
  • The phenotypic features of the skeletal muscle in a patient with this disorder are muscle glycogen depletion, mitochondrial proliferation, and a marked predominance of slow-twich, oxidative muscle fibres. (wikipedia.org)
  • Skeletal muscle - 15 g/kg wet wt. (powershow.com)
  • Skeletal muscle is affected, whereas the liver enzyme is normal. (powershow.com)
  • While some forms of GSD affect a single tissue type (for example, skeletal muscle in McArdle disease), others affect multiple systems. (iospress.com)
  • Within skeletal muscle and liver glucose is stored as glycogen. (wikipathways.org)
  • title=Regulation of glycogen synthase from mammalian skeletal muscle--a unifying view of allosteric and covalent regulation. (wikidoc.org)
  • What are the fates of glycogen in skeletal muscle? (greek.doctor)
  • Glycogen is found in largest amounts in the liver and in skeletal muscle, but is present in many other cell types, too, like the heart. (greek.doctor)
  • Skeletal muscle need a lot of energy while working, which is why this organ contains a lot of glycogen. (greek.doctor)
  • Skeletal muscle doesn't have glucose 6-phosphatase and therefore can't release the stored glycogen into the blood as glucose, and therefore can't help the liver maintain the blood glucose level. (greek.doctor)
  • Which of the following occurs during Glycogenolysis to maintain Tammy's blood glucose levels and fuel exercising skeletal muscle during this event? (mockdocs.org)
  • Enhanced Efficacy of Enzyme Replacement Therapy in Pompe Disease Through Mannose-6-Phosphate Receptor Expression in Skeletal Muscle. (sumobrain.com)
  • No limiting role for glycogenin in determining maximal attainable glycogen levels in rat skeletal muscle. (semanticscholar.org)
  • Bodily movement produced by the contraction of skeletal muscle that requires energy expenditure in excess of resting energy expenditure. (diabetesjournals.org)
  • Liver and skeletal muscle are the main storage organs. (amboss.com)
  • Glycogenolysis occurring in skeletal muscle could generate free glucose which could enter the blood stream. (pdfslide.net)
  • 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)
  • Analysis of skeletal muscle, heart, and liver found that GYS1 deficiency reduced polyglucosan body accumulation in all three tissues and PPP1R3C knockout reduced skeletal muscle polyglucosan bodies. (bvsalud.org)
  • On the other hand, much less is known about the structure of glycogen synthase, the key regulatory enzyme of glycogen synthesis. (wikipedia.org)
  • The crystal structure of glycogen synthase from Agrobacterium tumefaciens, however, has been determined at 2.3 A resolution. (wikipedia.org)
  • In the synthesis of glycogen, one ATP is required for every glucose unit incorporated into the polymeric branched structure of glycogen. (gsdhelp.info)
  • Structure of glycogen Figure 1. (gsdhelp.info)
  • The structure of glycogen is summarized in Figure 1 [2]. (gsdhelp.info)
  • Describe the structure of glycogen. (greek.doctor)
  • This figures shows the structure of glycogen. (biologydictionary.net)
  • The structure of glycogen consists of long polymer chains of glucose units connected by an alpha acetal linkage. (tdmuv.com)
  • However, missense mutation affecting some other residues of glycogenin-1 has also been shown to eliminate autoglycosilation. (wikipedia.org)
  • Similar to the glycogen synthase, the phosphorylase cannot remove glucose 4 residues from the end of one chain or branch. (wikibooks.org)
  • A. tumefaciens glycogen synthase (GS) purified to homogeneity from the above-mentioned cells was able to build its own primer by transferring Glc residues from ADP-Glc to an amino acid(s) in the same protein. (pnas.org)
  • Glycogen is a strongly branched polymer of α-D-glucose, with glucose residues in the linear chains linked by 1→4-bonds (~93% of the total number of bonds) and with branching after every 4-8 residues formed by 1→6-glycosidic bonds (~7% of the total number of bonds). (springer.com)
  • Glycogen is a very large, branched polymer of glucose residues. (wikipathways.org)
  • Debranching EnzymeThe activity of phosphorylase ceases 4 glucose residues from the branch point. (pdfslide.net)
  • During de novo synthesis, glucose molecules are added to tyrosine residues of primer protein glycogenin. (medicalbiochemist.com)
  • Its work will immediately come to a halt four residues away from α1-6 branch (which are exceedingly common in glycogen). (wikipedia.org)
  • Pyridoxal phosphate links with basic residues (in this case Lys680) and covalently forms a Schiff base . (wikipedia.org)
  • The function of liver glycogen is to maintain glucose homeostasis, generating glucose via glycogenolysis to compensate for the decrease of glucose levels that can occur between meals. (wikipedia.org)
  • When glycogen is broken down through a process called glycogenolysis, it is broken to make glucose which is then used by the human body or other organisms as energy. (wikibooks.org)
  • The breaking down of glycogen into glucose is called glycogenolysis. (wikibooks.org)
  • Glycogenolysis, process by which glycogen, the primary carbohydrate stored in the liver and muscle cells of animals, is broken down into glucose to provide. (gsdhelp.info)
  • Glycogenolysis takes place in the cells of muscle and liver tissues in response to hormonal and neural signals. (gsdhelp.info)
  • In muscle, glycogenolysis serves to provide an immediate source of glucosephosphate for glycolysis to provide energy for muscle contraction but not for other body tissues. (gsdhelp.info)
  • Glycogen is broken down via glycogenolysis into glucose-1-phosphate, which is converted to glucose and released into the bloodstream. (biologydictionary.net)
  • 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)
  • Hippocampal glycogen and glucose concentrations promptly decreased during repeated glucose deprivation, indicating that glycogenolysis does not fuel synaptic adaptation to repeated hypoglycemia. (diabetesjournals.org)
  • The phosphorylation sites of glycogen synthase are summarized below. (wikipedia.org)
  • Glycogen phosphorylase is also studied as a model protein regulated by both reversible phosphorylation and allosteric effects. (wikipedia.org)
  • increases glycogen phosphorylation as long as AMP levels are high). (pharmaceuticalintelligence.com)
  • Glycogen is a multi-branched polysaccharide. (wikipedia.org)
  • Glycogen is a sugar but in a polysaccharide form and is made up of many carbohydrates compared to that of glucose. (wikibooks.org)
  • However, the type and force of bonds connecting these proteins to the polysaccharide moiety of glycogen are significantly different. (springer.com)
  • Glycogen is a multi-branched polysaccharide of glucose that serves as an energy store primarily in muscle and liver. (gsdhelp.info)
  • These units are molecules of glucose, making glycogen a polysaccharide . (greek.doctor)
  • Glycogen is a large, branched polysaccharide that is the main storage form of glucose in animals and humans. (biologydictionary.net)
  • Glycogen is a polysaccharide consist of glucose linked together by glycosidic linkage. (medicalbiochemist.com)
  • Glycogen, the polysaccharide used by animals to store energy, is composed of alpha-1,4-glycosidic bonds with branched alpha-1,6 bonds present at about every tenth monomer. (wikibooks.org)
  • The formation of glycogen with glucose occurs through the help of the enzyme, glycogen synthase. (wikibooks.org)
  • Glycogen synthesis is carried out by the enzyme glycogen synthase in which the activated form of glucose, UDP-glucose (uridine diphosphate), is formed by way of the reaction between UTP and glucose-1 phosphate. (wikibooks.org)
  • Glycogen and starch synthesis involves elongation of α(1,4)-linked glucans by glycogen synthase (GS), an enzyme that uses UDP-glucose (Glc) or ADP-Glc as substrate donor in mammalian and fungal or in plant and bacterial cells, respectively ( 1 ). (pnas.org)
  • Glycogen is the analogue of starch, a less branched glucose polymer in plants, and is commonly referred to as animal starch, having a similar structure to amylopectin. (wikibooks.org)
  • Glycogen is an analogue of starch, which is the main form of glucose storage in most plants, but starch has fewer branches and is less compact than glycogen. (biologydictionary.net)
  • Glycogen is the storage form of glucose in animals and humans which is analogous to the starch in plants. (tdmuv.com)
  • Polysaccharides such as starch, glycogen, and dextrans are all stored in the liver and muscles to be converted to energy for later use. (wikibooks.org)
  • The starch molecules form a hollow helix that is suitable for easy energy access and storage. (wikibooks.org)
  • Starch, used by plant cells, is similar in structure but exists in two forms: amylose is the helical form of starch comprised only of alpha-1,4 linkages, and amylopectin has a structure like glycogen except that the branched alpha-1,6 linkages are present on only about one in 30 monomers. (wikibooks.org)
  • Typically each of the chains are formed with about 8-10 glucose molecules. (wikibooks.org)
  • Furthermore, glycogen is made up of many of these chains of glucose, where some glycogen molecules can contain up to some sources say 120,000 units of glucose while some sources say a range of 1,700 to 600,000. (wikibooks.org)
  • However, since glycogen synthase can only add glucose molecules, there is a protein by the name of glycogenin which acts like a primer and initially starts the reaction to form glycogen. (wikibooks.org)
  • Typically, up to about 10% of the mass of the liver can be composed of glycogen molecules. (wikibooks.org)
  • Glycogen is a branched-chain homopolysaccharide synthesized from α -D-glucose molecules [ 2 ]. (iospress.com)
  • The fourth carbon atom of the first unit of glucose is bound together with the first carbon atom of the glucose and so on, forming long chains of glucose molecules. (greek.doctor)
  • 4) glycosidic bonds as it attaches more and more glucose molecules to the glycogen. (greek.doctor)
  • While glucose and insulin levels are sufficiently high, glycogen chains are elongated by the addition of glucose molecules, a process termed glyconeogenesis. (biologydictionary.net)
  • These stored glycogen molecules can readily be degraded into glucose molecules and enter into the glycolytic pathway for energy. (medicalbiochemist.com)
  • Figure 2: Glycogen Synthase-addition of glucose from UDP-glucose to core glycogen molecules. (medicalbiochemist.com)
  • Figure 4: Glycogen phosphorylase-removal of glucose from glycogen molecules. (medicalbiochemist.com)
  • What starts the formation of glycogen and how many glucose molecules per chain in glycogen? (brainscape.com)
  • What are the two fates of the glucose 1-phosphate molecules from the glycogen? (brainscape.com)
  • In mammals, the major isozymes of glycogen phosphorylase are found in muscle, liver, and brain. (wikipedia.org)
  • How many isozymes of glycogen phosphorylase are there? (cram.com)
  • How can you maximize the activity of phosphorylase kinase? (cram.com)
  • Glycogen synthase is also regulated by protein phosphatase 1 (PP1), which activates glycogen synthase via dephosphorylation. (wikipedia.org)
  • it activates glycogen degredation at the same tome as it inhibits gycogen synthesis. (cram.com)
  • What enzyme activates glycogen phosphorylase? (cram.com)
  • Therefore, the liver has a higher amount of glycogen per unit mass of tissue. (wikibooks.org)
  • However, the total mass of the muscle in the body is greater than that of the liver, and so the total amount of glycogen will exceed that of the total amount of glycogen in the liver. (wikibooks.org)
  • The amount of glycogen in a liver can maintain the blood glucose level for 1 day of fasting - after this point the glycogen stores are depleted, and so glucose only originates from gluconeogenesis. (greek.doctor)
  • However, given the greater mass of muscle in the body, the total amount of glycogen stored in muscle is greater than that stored in liver. (biologydictionary.net)
  • A Glycogenin-1 deficiency leads to Glycogen storage disease type XV. (wikipedia.org)
  • Deficiency of glycogenin-1 is detected in the sequence of the glycogenin-1 gene, GYG1, which revealed a non-sense mutation in one allele and a missense mutation, Thr83Met, in the other. (wikipedia.org)
  • Triheptanoin's Effect on Fatty Acid Oxidation and Exercise Tolerance in Patients With Debrancher Deficiency, Glycogenin-1 Deficiency and Phosphofructoinase Deficiency at Rest and During Exercise. (clinicaltrials.gov)
  • Glycogen is made up of chains of glucose. (wikibooks.org)
  • Inability to break the glucose free from the glycogen chains. (mockdocs.org)
  • Glycogen phosphorylase can act only on linear chains of glycogen (α1-4 glycosidic linkage). (wikipedia.org)
  • These beta 1,4-glycosidic bonds, unlike the alpha 1,4 glycosidic bonds, force celullose to form long and sturdy straight chains that can interact with one another through hydrogen bonds to form fibers. (wikibooks.org)
  • Glycogenin is an oligomer, and it's capable of interacting with several proteins. (wikipedia.org)
  • In recent years, a family of proteins has been identified, the GNIPs (glycogenin-interacting protein), that interacts with glycogenin stimulating its autoglycolsilation activity. (wikipedia.org)
  • It was concluded that, contrary to what happens in mammalian and yeast cells in which two different proteins are required for linear α(1,4)-linked glucan formation (glycogenin for initiation and GS for further elongation), in A. tumefaciens and probably in all other bacteria, the same protein is involved in both glycogen initiation and elongation. (pnas.org)
  • Cyclic AMP binding to the regulatory subunit of protein kinase A causes the catalytic subunit of PKA to dissociate from the regulatory subunit enabling it to phosphorylate other proteins including glycogen phosphorylase b. (gsdhelp.info)
  • The protein glycogenin, which is involved in glycogen synthesis, is located at the core of each glycogen granule. (biologydictionary.net)
  • Specifically, The inter-subunit interactions are mediated by the α15/16 helix pairs, forming allosteric sites between subunits in one combination of dimers and active sites between subunits in the other combination of dimers. (wikipedia.org)
  • Ca+ inds to what subunit of the phosphorylase kinase enzyme? (cram.com)
  • APBD mice were crossed with mice deficient in glycogen synthase (GYS1), or mice deficient in protein phosphatase 1 regulatory subunit 3C (PPP1R3C), a protein involved in the activation of GYS1. (bvsalud.org)
  • It has been established that another enzyme, glycogenin, participates in the initiation of yeast and mammalian cell glycogen synthesis ( 2 , 3 ). (pnas.org)
  • Nonetheless, a more recent characterization of the Saccharomyces cerevisiae (yeast) glycogen synthase crystal structure reveals that the dimers may actually interact to form a tetramer. (wikipedia.org)
  • Nutrient limitation (low levels of carbon, phosphorus, nitrogen, or sulfur) can stimulate glycogen formation in yeast, while bacteria synthesize glycogen in response to readily available carbon energy sources with limitation of other nutrients. (biologydictionary.net)
  • Bacterial growth and yeast sporulation have also been associated with glycogen accumulation. (biologydictionary.net)
  • Insulin also activates enzymes involved in glycogen synthesis, such as glycogen synthase. (biologydictionary.net)
  • Glycogen synthase catalyzes the conversion of the glucosyl (Glc) moiety of uridine diphosphate glucose (UDP-Glc) into glucose to be incorporated into glycogen via an α(1→4) glycosidic bond. (wikipedia.org)
  • Glycogen involves two types of glycosidic linkage: In muscle, but not liver cells, the glucose is subsequently gllycogenolysis to glucosephosphate by a fifth enzyme hexokinase and enters the glycolytic pathway [4]. (gsdhelp.info)
  • 6) glycosidic bond is formed, which creates a branch. (greek.doctor)
  • then, the enzyme cleaves the alpha-1,6-glycosidic linkage to yeild free glucose + debranched glycogen. (cram.com)
  • It is formed by mostly alpha 1,4 glycosidic linkages but branching occurs more frequently than in amylopectin as alpha 1,6 glycosidic linkages occur about every ten units. (wikibooks.org)
  • The two types of glycosidic bonds (alpha-1,4 and alpha-1,6) in glycogen are shown. (wikibooks.org)
  • Because dietary intake of glucose (and glucose precursors) is sporadic and gluconeogenesis cannot occur in rapid response to falling blood glucose levels, glycogen serves as a means for storing glucose in a form that can be readily mobilized. (iospress.com)
  • The glucose in the form of glucosephosphate is synthesized directly from glucose or as the end product of gluconeogenesis. (gsdhelp.info)
  • After gluconeogenesis, glucose is converted from glycogen in liver or muscle for glycolysis. (scribd.com)
  • Other monosaccharides are also made to G6-P via gluconeogenesis, then glucose can be stored as glycogen. (scribd.com)
  • This glucose originates mostly from the breakdown of glycogen, but also from gluconeogenesis. (greek.doctor)
  • It is stored in the form of granules in the cytoplasm of the cell and is the main storage form of glucose in the body. (gsdhelp.info)
  • Relative to sleep, all wake conditions increased the number of glycogen granules around the synapses to a similar extent. (frontiersin.org)
  • However, progressively longer periods of wake were associated with progressively smaller glycogen granules, suggesting increased turnover. (frontiersin.org)
  • Finally, chronic sleep restriction moved glycogen granules closer to the synaptic cleft. (frontiersin.org)
  • Glycogen is hydrated with three to four parts water and forms granules in the cytoplasm that are 10-40nm in diameter. (biologydictionary.net)
  • What happens then in new cells to make now glycogen granules? (tdmuv.com)
  • Thus glycogen granules have a protein core. (tdmuv.com)
  • Glycogen is the most important carbohydrate storage medium in the human body, found in cytosolic granules. (amboss.com)
  • Polymers thus generated are substrates of an enzyme that introduces α(1,6) branches to yield the characteristic glycogen and amylopectin structures. (pnas.org)
  • Schematic two-dimensional cross-sectional view of glycogen: A core protein of glycogenin is surrounded by branches of glucose units. (gsdhelp.info)
  • The branches are formed by linking C # 1 to a C # 6 through an acetal linkages. (tdmuv.com)
  • In glycogen, the branches occur at intervals of 8-10 glucose units, while in amylopectin the branches are separated by 12-20 glucose units. (tdmuv.com)
  • Although it encodes all enzymes known to be required for glycogen synthesis and utilization, the cluster does not encode a protein displaying significant similarity to glycogenin, nor does the entire A. tumefaciens genome code for such a protein ( 6 ). (pnas.org)
  • It is capable of self-glucosylation, forming an oligosaccharide primer that serves as a substrate for glycogen synthase. (wikipedia.org)
  • In this report we present evidence indicating that A. tumefaciens GS not only elongates α(1,4)-linked glucans but also forms the primer required for the elongation process by catalyzing its own glucosylation. (pnas.org)
  • However, since glycogen synthase requires an oligosaccharide primer as a glucose acceptor, it relies on glycogenin to initiate de novo glycogen synthesis. (wikipedia.org)
  • Note also that glycogen synthase requires a 'primer. (tdmuv.com)
  • Can use a special primer protein (glycogenin). (tdmuv.com)
  • Glycogen is synthesized from glucose when blood glucose levels are high, and serves as a ready source of glucose for tissues throughout the body when blood glucose levels decline. (biologydictionary.net)
  • When blood glucose levels fall below a certain level, glucagon released from the pancreas signals to liver cells to break down glycogen. (biologydictionary.net)
  • Thus, glycogen serves as the main buffer of blood glucose levels by storing glucose when it levels are high and releasing glucose when levels are low. (biologydictionary.net)
  • Instead, muscular glycogen provides energy for the muscles themselves. (greek.doctor)
  • Each of these protein kinases lead to phosphorylated and catalytically inactive glycogen synthase. (wikipedia.org)
  • Activated PP1 directly glycogenwsis glycogen phosphorylase a, reforming the inactive glycogen phosphorylase b, whereas phosphodiesterase converts cAMP to AMP, thus inactivating PKA and its ability to phosphorylate activate glycogen phosphorylase. (gsdhelp.info)
  • Glycogenin catalyzes glucosylation of a Tyr residue in the same protein by using the same sugar donors as that used by GS for further chain elongation. (pnas.org)
  • For enzymes in the GT3 family, these regulatory kinases inactivate glycogen synthase by phosphorylating it at the N-terminal of the 25th residue and the C-terminal of the 120th residue. (wikipedia.org)
  • A point mutation in glycogenin causes a stop codon to form where a tyrosine residue should be. (mockdocs.org)
  • 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)
  • What are the allosteric regulators of glycogen phosphorylase? (cram.com)
  • Glycogen is typically more concentrated in the liver, but because humans have much more muscle mass, our muscles store about three quarters of the total glycogen in our body. (wikipedia.org)
  • Glycogen synthase is directly regulated by glycogen synthase kinase 3 (GSK-3), AMPK, protein kinase A (PKA), and casein kinase 2 (CK2). (wikipedia.org)
  • Protein kinase phosphorylates the alpha and beta subunits of phosphorylase kinase to activate it. (cram.com)
  • This led to the discovery of cAMP activated protein kinase and the assembly of a very complex system in the glycogen granule that is not a simple carbohydrate polymer. (pharmaceuticalintelligence.com)
  • As glucose and insulin levels decrease, glycogen synthesis ceases. (biologydictionary.net)
  • When insulin is present is inactivates GSK3 and activates PP1 where glycogen synthase is activated. (brainscape.com)
  • However, muscle-specific glycogen synthase activation may lead to excessive accumulation of glycogen, leading to damage in the heart and central nervous system following ischemic insults. (wikipedia.org)
  • Glycogen storage disease type 4 (GSD 4) is part of a group of disorders which lead to abnormal accumulation of glycogen (a storage form of glucose) in various parts of the body. (diseaseinfosearch.org)
  • Structurally, glycogen is very similar to amylopectin with alpha acetal linkages, however, it has even more branching and more glucose units are present than in amylopectin. (tdmuv.com)
  • Another one of the important functions of glycogen is that it helps maintain glucose levels in the body. (wikibooks.org)
  • Glycogenin-1 is an enzyme that is involved in the biosynthesis of glycogen. (wikipedia.org)
  • This Review contains major "Glossary of Biochemistry Stubs"- related terms, short phrases and links grouped together in the form of Encyclopedia article. (keywen.com)
  • In muscle, what does it produce for entry into glycolysis? (cram.com)
  • The enzymes which synthesize and break down glycogen can only act on non-reducing ends. (greek.doctor)
  • Acid alpha-glucosidase , also called α-1,4-glucosidase [5] and acid maltase , [6] is an enzyme ( EC 3.2.1.20 ) that helps to break down glycogen in the lysosome . (wikipedia.org)
  • Glycogen homeostasis is a highly regulated process that allows the body to store or release glucose depending on its energetic needs. (biologydictionary.net)
  • However, glycogen is different because it is a storage form of energy. (wikibooks.org)
  • In a recent study of transgenic mice, an overexpression of glycogen synthase and an overexpression of phosphatase both resulted in excess glycogen storage levels. (wikipedia.org)
  • Liver glycogen serves as a storage pool to maintain the blood glucose level during fasting, whereas muscle glycogen synthesis accounts for disposal of up to 90% of ingested glucose. (wikipedia.org)
  • Within the last decade, DNA mutation analysis has become the primary method for diagnosing glycogen storage disease. (iospress.com)
  • Astrocytic glycogen represents the only form of glucose storage in the brain, and one of the outcomes of its breakdown is the production of lactate that can be used by neurons as an alternative energetic substrate. (frontiersin.org)
  • Glycogen is also an important form of glucose storage in fungi and bacteria . (biologydictionary.net)
  • Microorganisms possess mechanisms for storing energy to cope in the event of limited environmental resources, and glycogen represents a main energy storage form. (biologydictionary.net)
  • The invention provides compositions and methods for treating glycogen storage diseases or conditions with a buildup of glycogen. (sumobrain.com)
  • Cyclic AMP elevator compositions are shown to reduce glycogen storage in affected cells and therefore can be used to treat or reduce symptoms in subjects having glycogen storage diseases or conditions with a buildup of glycogen. (sumobrain.com)
  • 1. A method of treating a glycogen storage disease or a condition with a buildup of glycogen in a subject in need of such treatment comprising administering a therapeutically effective amount of a cyclic AMP elevator to the subject in need of treatment for a glycogen storage disease. (sumobrain.com)
  • 14. The method of any one of the preceding claims, further comprising administering a therapeutically effective amount of an enzyme replacement therapy, a gene therapy, a chaperone therapy, a substrate reduction therapy, or combinations thereof to the subject with a glycogen storage disease or a condition with a buildup of glycogen. (sumobrain.com)
  • Any glucose in excess of the needs for energy and storage as glycogen is converted to fat. (tdmuv.com)
  • This crevice connects the glycogen storage site to the active, catalytic site. (wikipedia.org)
  • Following organizations serve the condition "Glycogen Storage Disease Type 4" for support, advocacy or research. (diseaseinfosearch.org)
  • Finding the right clinical trial for Glycogen Storage Disease Type 4 can be challenging. (diseaseinfosearch.org)
  • The terms "Glycogen Storage Disease Type 4" returned 1 free, full-text research articles on human participants. (diseaseinfosearch.org)
  • Novel mutations in the gene encoding acid α-1,4-glucosidase in a patient with late-onset glycogen storage disease type II (Pompe disease) with impaired intelligence. (diseaseinfosearch.org)
  • source of ATP (pyruvate) energy storage (glycogen) molecular precursor (ECM and cell wall polysacchs. (brainscape.com)
  • What does the variance is glycogen storage disease suggest? (cram.com)
  • OBJECTIVE: Adult polyglucosan body disease (APBD) is an adult-onset neurological variant of glycogen storage disease type IV. (bvsalud.org)
  • These findings represent a critical step toward the development of a treatment for APBD and potentially other glycogen storage disease type IV patients. (bvsalud.org)
  • [6] Errors in this gene cause glycogen storage disease type II (Pompe disease). (wikipedia.org)
  • Defects in this gene are the cause of glycogen storage disease II , also known as Pompe disease, which is an autosomal recessive disorder with a broad clinical spectrum. (wikipedia.org)
  • After a meal the blood glucose level is very high, but it quickly normalizes as the tissues of the body take in the glucose, both for energy consumption and to store it in the form of glycogen. (greek.doctor)
  • In addition to liver and muscle, glycogen in found in smaller amounts in other tissues, including red blood cells, white blood cells, kidney cells, and some glial cells . (biologydictionary.net)
  • The first observation arrived from a sample due to be studied on the following day of glycogen synthetase. (pharmaceuticalintelligence.com)
  • In the diabetic rats, high glucose levels and a decrease in hepatic glycogen contents could be attributed to less availability of the active form of the enzyme glycogen synthetase (Bollen and Stalmans 1984). (thefreedictionary.com)
  • Glycogen synthetase from rat liver: (Glucose)n +(UDPG) [right arrow] or [vector] (Glucose)n + 1 + UDP. (thefreedictionary.com)
  • what, self-glycosylating, protein forms the initial glucose monomer chain from which glycogen can be formed? (cram.com)
  • 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)
  • In contrary, glycogen breakdown release glucose for muscle contraction and regulation of blood glucose. (medicalbiochemist.com)
  • Although the catalytic mechanisms used by glycogen synthase are not well known, structural similarities to glycogen phosphorylase at the catalytic and substrate binding site suggest that the mechanism for synthesis is similar in glycogen synthase and glycogen phosphorylase. (wikipedia.org)
  • phosphorylase b is inactivated and is not phosphorylated but glucagon and epinephrine activate phosphorylase and lead to it being phosphorylated and activated. (brainscape.com)
  • The mutations in the glycogenin-1 gene GYG1 are also a cause of cardiomyopathy and arrhythmia. (wikipedia.org)
  • Glycogen synthesis is, unlike its breakdown, endergonic. (wikipathways.org)
  • In liver cells the main purpose of the breakdown of glycogen is for the release of glucose into the bloodstream for uptake by other cells. (gsdhelp.info)
  • Which enzymes are involved in the breakdown of glycogen? (greek.doctor)
  • Glycogen breakdown in the liver is critical for supplying glucose to meet the body's energetic needs. (biologydictionary.net)
  • The glucose-1-phosphate produced from glycogen breakdown in muscle fibers is converted to glucose-6-phosphate and provides energy to the muscle during a bout of exercise or in response to stress, as in the fight-or-flight response. (biologydictionary.net)
  • Phosphorylase was originally thought to be the synthetic as well as breakdown enzyme since the reaction is readily reversible in vitro . (tdmuv.com)
  • The glycogen synthesis and breakdown pathway are highly regulated and, the synthesis and breakdown do not occur at the same time. (medicalbiochemist.com)
  • The glycogen synthesis and breakdown are reciprocally regulated to ensure that both pathways do not occur at the same time in the cell. (medicalbiochemist.com)
  • Standard studies performed by the pathologist include muscle or liver histology findings in combination with electron microscopy and enzyme studies. (iospress.com)
  • Since most energy in the brain is used to sustain synaptic activity, here we employed tridimensional electron microscopy to quantify glycogen content in the astrocytic processes surrounding the synapse. (frontiersin.org)
  • 20 - 40 β-particles form a so-called α-rosette , which can be visualized on electron microscopy. (greek.doctor)
  • Glycogenin-linked maltooctasaccharides thus formed are used as primers by GS, and apparently glycogenin remains linked to the polymer through the entire elongation process ( 2 , 3 ). (pnas.org)