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glykogenóza typu II

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)
MSH

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, heart, liver, spinal cord, and brain; three forms have been described: the infantile form is fatal in infancy and presents with hypotonia and a hypertrophic cardiomyopathy; 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.
CSP

An autosomal recessive inherited type of glycogen storage disease caused by deficiency of the enzyme acid alpha-glucosidase. It results in the abnormal accumulation of glycogen in the heart, skeletal muscles, liver, and nervous system.
NCI

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glykogenóza typu III

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.
MSH

autosomal recessive metabolic disorder due to deficient expression of amylo-1,6-glucosidase; clinical course 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.
CSP

An autosomal recessive inherited type of glycogen storage disease caused by deficiency of the glycogen debranching enzyme. It results in the accumulation of structurally abnormal glycogen in the heart, skeletal muscles, and/or liver.
NCI

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glykogenóza typu IV

An autosomal recessive metabolic disorder due to a deficiency in expression of glycogen branching enzyme 1 (alpha-1,4-glucan-6-alpha-glucosyltransferase), resulting in an accumulation of abnormal GLYCOGEN with long outer branches. Clinical features are MUSCLE HYPOTONIA and CIRRHOSIS. Death from liver disease usually occurs before age 2.
MSH

autosomal recessive metabolic disorder due to a deficiency in expression of branching enzyme (alpha-1,4-glucan-6-alpha-glucosyltransferase), resulting in an accumulation of abnormal glycogen with long outer branches; clinical features are muscle hypotonia and cirrhosis; death from liver disease usually occurs before age 2.
CSP

A rare inherited type of glycogen storage disease caused by deficiency of amylo-1,4-1,6 transglucosidase.
NCI

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glykogenóza typu V

Glycogenosis due to muscle phosphorylase deficiency. Characterized by painful cramps following sustained exercise.
MSH

glycogenosis due to muscle phosphorylase deficiency; characterized by painful cramps following sustained exercise.
CSP

An autosomal recessive inherited type of glycogen storage disease caused by deficiency of myophosphorylase. It results in myalgias, muscle cramping and stiffness, and exercise intolerance.
NCI

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glykogenóza typu VI

A hepatic GLYCOGEN STORAGE DISEASE in which there is an apparent deficiency of hepatic phosphorylase (GLYCOGEN PHOSPHORYLASE, LIVER FORM) activity.
MSH

hepatic glycogen storage disease in which there is an apparent deficiency of hepatic phosphorylase activity.
CSP

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glykogenóza typu VII

An autosomal recessive glycogen storage disease in which there is deficient expression of 6-phosphofructose 1-kinase in muscle (PHOSPHOFRUCTOKINASE-1, MUSCLE TYPE) resulting in abnormal deposition of glycogen in muscle tissue. These patients have severe congenital muscular dystrophy and are exercise intolerant.
MSH

autosomal recessive glycogen storage disease in which there is deficient expression of 6-phosphofructose 1-kinase in muscle resulting in abnormal deposition of glycogen in muscle tissue; patients have severe congenital muscular dystrophy and are exercise intolerant.
CSP

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glykogen – nemoc z ukládání typ VIII

An x-linked recessive hepatic glycogen storage disease resulting from lack of expression of phosphorylase-b-kinase activity. Symptoms are relatively mild; hepatomegaly, increased liver glycogen, and decreased leukocyte phosphorylase are present. Liver shrinkage occurs in response to glucagon.
MSH

x-linked recessive hepatic glycogen storage disease resulting from lack of expression of phosphorylase-b-kinase activity; symptoms are relatively mild, hepatomegaly, increased liver glycogen, and decreased leukocyte phosphorylase are present; liver shrinkage occurs in response to glucagon.
CSP

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glykogensynthasa

An enzyme that catalyzes the transfer of D-glucose from UDPglucose into 1,4-alpha-D-glucosyl chains. EC 2.4.1.11.
MSH

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glykogensynthetasofosfatasa

An enzyme that catalyzes the conversion of phosphorylated, inactive glycogen synthase D to active dephosphoglycogen synthase I. EC 3.1.3.42.
MSH

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Glycol

compound containing two hydroxyl groups.
CSP

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glykoláty

hydroxyacetic acid or its salts or esters; an intermediate in the conversion of serine to glycine.
CSP

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glykolipidy

Any compound containing one or more monosaccharide residues bound by a glycosidic linkage to a hydrophobic moiety such as an acylglycerol (see GLYCERIDES), a sphingoid, a ceramide (CERAMIDES) (N-acylsphingoid) or a prenyl phosphate. (From IUPAC`s webpage)
MSH

compound containing one or more monosaccharide residues bound by a glycosidic linkage to a hydrophobic moiety such as an acylglycerol, a sphingoid, a ceramide, or a prenyl phosphate.
CSP

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glykoly

A generic grouping for dihydric alcohols with the hydroxy groups (-OH) located on different carbon atoms. They are viscous liquids with high boiling points for their molecular weights.
MSH

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glykolýza

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.
MSH

called the Embden-Meyerhoff or glycolytic pathway in which glucose is anaerobically catabolized into the simpler compounds lactic acid or pyruvic acid, resulting in energy stored in the form of ATP.
CSP

A process in which glucose (sugar) is partially broken down by cells in enzyme reactions that do not need oxygen. Glycolysis is one method that cells use to produce energy. When glycolysis is linked with other enzyme reactions that use oxygen, more complete breakdown of glucose is possible and more energy is produced.
NCI

The chemical reactions and pathways resulting in the breakdown of a monosaccharide (generally glucose) into pyruvate, with the concomitant production of a small amount of ATP. Pyruvate may be converted to ethanol, lactate, or other small molecules, or fed into the TCA cycle. [ISBN:0716720094]
GO

A series of anaerobic chemical reactions that cells utilize to produce energy. Glycolysis is a biochemical pathway in which glucose is catabolized into lactate or pyruvate via enzymatic reactions to generate ATP.
NCI

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glykopeptidy

Proteins which contain carbohydrate groups attached covalently to the polypeptide chain. The protein moiety is the predominant group with the carbohydrate making up only a small percentage of the total weight.
MSH

peptides with covalently attached carbohydrate, usually resulting from biodegradation or laboratory hydrolysis of glycoproteins.
CSP

Peptides that contain carbohydrate moieties (glycans) covalently attached to the side chains of the amino acid residues that constitute the peptide. (Wikipedia)
NCI

A short chain of amino acids (the building blocks of proteins) that has sugar molecules attached to it. Some glycopeptides have been studied for their ability to stimulate the immune system.
NCI

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glykoforin

The major sialoglycoprotein of the human erythrocyte membrane. It consists of at least two sialoglycopeptides and is composed of 60% carbohydrate including sialic acid and 40% protein. It is involved in a number of different biological activities including the binding of MN blood groups, influenza viruses, kidney bean phytohemagglutinin, and wheat germ agglutinin.
MSH

family of acidic integral membrane glycoproteins whose sialate groups impart a net negative charge to erythrocytes.
CSP

A family of sialoglycoproteins that are found in erythrocytes. Members of this family are transmembrane proteins and are able to interact with both oligosaccharides on the extracellular surface and the cytoplasmic contractile proteins spectrin and actin.
NCI

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Glycophorin A

Glycophorins A (GYPA) and B (GYPB) are major sialoglycoproteins of the human erythrocyte membrane which bear the antigenic determinants for the MN and Ss blood groups. In addition to the M or N and S or s antigens, that commonly occur in all populations, about 40 related variant phenotypes have been identified. These variants include all the variants of the Miltenberger complex and several isoforms of Sta; also, Dantu, Sat, He, Mg, and deletion variants Ena, S-s-U- and Mk. Most of the variants are resulted from gene recombinations between GYPA and GYPB. (from RefSeq)
NCI

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beta-N-acetylglukosaminylglykopeptid-beta-1,4-galaktosyltransferasa

An enzyme that catalyzes the transfer of galactose from UDP-galactose to a specific glycoprotein receptor, 2-acetamido-2-deoxy-D-glucosyl-glycopeptide, during glycopeptide synthesis. EC 2.4.1.38.
MSH

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glykoproteinové hormony alfa subjednotkové

The alpha chain of pituitary glycoprotein hormones (THYROTROPIN; FOLLICLE STIMULATING HORMONE; LUTEINIZING HORMONE) and the placental CHORIONIC GONADOTROPIN. Within a species, the alpha subunits of these four hormones are identical; the distinct functional characteristics of these glycoprotein hormones are determined by the unique beta subunits. Both subunits, the non-covalently bound heterodimers, are required for full biologic activity.
MSH

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trombocytový glykoproteinový komplex Ib-IX

Platelet membrane glycoprotein complex essential for normal platelet adhesion and clot formation at sites of vascular injury. It is composed of three polypeptides, GPIb alpha, GPIb beta, and GPIX. Glycoprotein Ib functions as a receptor for von Willebrand factor and for thrombin. Congenital deficiency of the GPIb-IX complex results in Bernard-Soulier syndrome. The platelet glycoprotein GPV associates with GPIb-IX and is also absent in Bernard-Soulier syndrome.
MSH

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glykoproteiny

Conjugated protein-carbohydrate compounds including mucins, mucoid, and amyloid glycoproteins.
MSH

ubiquitous family of proteins with covalently attached oligosaccharide side chains which impart unique properties of solubility, size, antigenicity, ligand affinity, cellular targetting, and stability.
CSP

A protein that has sugar molecules attached to it.
NCI

A conjugated protein having a carbohydrate component.
NCI

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glykopyrolát

A muscarinic antagonist used as an antispasmodic, in some disorders of the gastrointestinal tract, and to reduce salivation with some anesthetics.
MSH

A synthetic quaternary ammonium anticholinergic agent with antimuscarinic activity. Glycopyrrolate binds to peripheral muscarinic receptors and inhibits cholinergic transmission present in the autonomic effector cells of smooth muscle, cardiac muscle, the sinoatrial node, the atrioventricular node, exocrine glands, and in the autonomic ganglia. As a result, the volume and free acidity of gastric secretions and excessive pharyngeal, tracheal, and bronchial secretions are reduced.
NCI

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glykosaminoglykany

Heteropolysaccharides which contain an N-acetylated hexosamine in a characteristic repeating disaccharide unit. The repeating structure of each disaccharide involves alternate 1,4- and 1,3-linkages consisting of either N-acetylglucosamine or N-acetylgalactosamine.
MSH

polymers of alternating sugar acids and aminosugars, which may be sulfated or otherwise further substituted; contribute to adhesion, elasticity, viscosity, etc., of extracellular substances.
CSP

A type of long, unbranched polysaccharide molecule. Glycosaminoglycans are major structural components of cartilage and are also found in the cornea of the eye.
NCI

Linear polysaccharides composed of disaccharide repeating units of glycosamine-glycans, two monosaccharide units (hexosamine and the repeating disaccharide of either hexuronate or galactose) located on the surface of cells or in the extracellular matrix contributing to adhesion, elasticity, and viscosity of extracellular substances.
NCI

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glykosidhydrolasy

covering the sugar hydrolases.
CSP

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glykosidy

Any compound that contains a constituent sugar, in which the hydroxyl group attached to the first carbon is substituted by an alcoholic, phenolic, or other group. They are named specifically for the sugar contained, such as glucoside (glucose), pentoside (pentose), fructoside (fructose), etc. Upon hydrolysis, a sugar and nonsugar component (aglycone) are formed. (From Dorland, 28th ed; From Miall`s Dictionary of Chemistry, 5th ed)
MSH

any compound that contains a constituent sugar, in which the hydroxyl group attached to the first carbon is substituted by an alcoholic, phenolic, or other group.
CSP

An ester containing a sugar component (glycol) and a nonsugar (aglycone) component attached via oxygen or nitrogen bond. Hydrolysis of a glycoside yields one or more sugars.
NCI

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glykosfingolipidy

Lipids containing at least one monosaccharide residue and either a sphingoid or a ceramide (CERAMIDES). They are subdivided into NEUTRAL GLYCOSPHINGOLIPIDS comprising monoglycosyl- and oligoglycosylsphingoids and monoglycosyl- and oligoglycosylceramides; and ACIDIC GLYCOSPHINGOLIPIDS which comprises sialosylglycosylsphingolipids (GANGLIOSIDES); SULFOGLYCOSPHINGOLIPIDS (formerly known as sulfatides), glycuronoglycosphingolipids, and phospho- and phosphonoglycosphingolipids. (From IUPAC`s webpage)
MSH

spingolipid in which the head group is a mono or oligosaccharide unit.
CSP

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glykosurie

The appearance of an abnormally large amount of GLUCOSE in the urine, such as more than 500 mg/day in adults. It can be due to HYPERGLYCEMIA or genetic defects in renal reabsorption (RENAL GLYCOSURIA).
MSH

presence of glucose in the urine, especially the excretion of an abnormally large amount of sugar (glucose) in the urine.
CSP

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DIABETES RENALA

An autosomal inherited disorder due to defective reabsorption of GLUCOSE by the PROXIMAL RENAL TUBULES. The urinary loss of glucose can reach beyond 50 g/day. It is attributed to the mutations in the SODIUM-GLUCOSE TRANSPORTER 2 encoded by the SLC5A2 gene.
MSH

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glykosylace

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.
MSH

chemical or biochemical addition of carbohydrate or glycosyl groups to other chemicals, especially peptides or proteins.
CSP

The covalent attachment of a glycosyl residue to a substrate molecule. [GOC:mah]
GO

The covalent chemical or biochemical addition of carbohydrate or glycosyl groups to other chemicals, by glycosyl transferases.
NCI

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glycin-tRNA-ligasa

An enzyme that activates glycine with its specific transfer RNA. EC 6.1.1.14.
MSH

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