72,234 articles
Glycine tabacina
Glycine tabacina (Variable Glycine), a perennial Fabaceae herb and wild soybean relative native from southern China and East Asia to Australia and the western Pacific; diploid B2B2 genome, 2n=40, with an allotetraploid complex and multiple polyploid origins.
Glycine transporter
Glycine transporters GlyT1 (SLC6A9) and GlyT2 (SLC6A5) are SLC6 Na+/Cl−-dependent reuptake proteins regulating inhibitory glycinergic transmission and NMDA receptor co-activation; drug targets in schizophrenia, hyperekplexia, and pain.
Glycine-tRNA ligase, beta subunit
Beta subunit of bacterial α2β2 glycyl-tRNA synthetase: ~65 kDa chain carrying tRNA recognition elements, encoded with the 303-codon alpha chain on one E. coli mRNA, evolutionarily derived from alanyl-tRNA synthetase.
Glycoamylase-like, conserved domain
Glycoamylase-like conserved domain: the GH15 glucoamylase catalytic domain, an inverting (α/α)6-barrel exo-hydrolase (EC 3.2.1.3) releasing β-glucose from starch, with Glu acid/base catalysis and an O-glycosylated linker to a starch-binding domain.
Glycobiology
Glycobiology: the study of glycan (complex carbohydrate) structure, biosynthesis, and function; term coined 1988; dedicated journal Glycobiology (OUP) since 1990; central challenge is linking glycan structure to function.
Glycocalyx
The glycocalyx, also known as the cell coat or pericellular matrix, is a carbohydrate-rich layer coating the outer surface of most cells, named in 1963 by H. Stanley Bennett.
Glycocholic Acid
Glycocholic acid, also called cholylglycine, is a conjugated bile acid in which cholic acid is linked to glycine; in mammalian bile it solubilizes fats for absorption.
Glycogen
Glycogen, also called animal starch, is the branched α-glucose polymer storing carbohydrate in animals, fungi, and bacteria, mainly in liver and skeletal muscle, with α(1→4) chains and α(1→6) branches every 8–12 residues; defects cause glycogen storage diseases.
Glycogen debranching enzyme
Glycogen debranching enzyme (GDE, AGL gene product; amylo-1,6-glucosidase, 4-α-glucanotransferase) is a bifunctional enzyme that removes glycogen branch points during glycogenolysis; its deficiency causes glycogen storage disease type III (Cori/Forbes disease).
Glycogen metabolism
Glycogen metabolism: synthesis (glycogenesis) and breakdown (glycogenolysis) of glycogen, the branched glucose polymer stored mainly in liver and muscle, regulated by insulin and glucagon and linked to glycogen storage diseases.
Glycogen storage disease due to aldolase A deficiency
Glycogen storage disease due to aldolase A deficiency (GSD type XII, GSD12, OMIM 611881) is an ultra-rare autosomal recessive ALDOA disorder causing hemolytic anemia, fever-triggered rhabdomyolysis, and learning disabilities, with no specific treatment.
Glycogen storage disease due to glucose-6-phosphatase deficiency
Glycogen storage disease due to glucose-6-phosphatase deficiency (GSD I, von Gierke disease): autosomal recessive disorder of G6PC or SLC37A4 causing fasting hypoglycemia, hepatomegaly, treated with cornstarch and glucose regimens.
Glycogen storage disease due to glycogen branching enzyme deficiency
Glycogen storage disease type IV, Andersen disease, amylopectinosis: autosomal recessive GBE1 (3p12) deficiency causing polyglucosan accumulation; hepatic, neuromuscular, cardiac continuum from fetal onset to adult polyglucosan body disease.
Glycogen storage disease due to hepatic glycogen synthase deficiency
Glycogen storage disease type 0a (GSD 0a, hepatic glycogen synthase deficiency, GYS2, OMIM 240600): rare autosomal recessive liver glycogen synthesis defect causing fasting ketotic hypoglycemia without hepatomegaly and postprandial hyperglycemia.
Glycogen storage disease due to liver and muscle phosphorylase kinase deficiency
Glycogen storage disease due to liver and muscle phosphorylase kinase deficiency, also called GSD IXb or PHKB-related GSD IX, is an autosomal recessive glycogenolysis defect causing hepatomegaly, hypoglycemia, and mild muscle involvement, managed with diet and uncooked cornstarch.
Glycogen storage disease due to liver glycogen phosphorylase deficiency
Glycogen storage disease type VI, also called Hers disease, is a rare autosomal recessive liver glycogenosis caused by PYGL deficiency of hepatic glycogen phosphorylase, causing hepatomegaly, growth delay, and ketotic hypoglycemia.
Glycogen storage disease due to liver phosphorylase kinase deficiency
Liver phosphorylase kinase deficiency (GSD IXa/IXb/IXc; formerly GSD VIa, VIII) is an inherited glycogen storage disease causing hepatomegaly, fasting hypoglycemia, and elevated transaminases; genes PHKA2, PHKB, PHKG2.
Glycogen storage disease due to muscle beta-enolase deficiency
Glycogen storage disease type XIII (GSDXIII, GSD13, muscle enolase deficiency) is an extremely rare autosomal-recessive ENO3 metabolic myopathy causing exercise intolerance, myalgia, and rhabdomyolysis from muscle beta-enolase deficiency.
Glycogen storage disease due to muscle glycogen phosphorylase deficiency
Glycogen storage disease type V, McArdle disease, myophosphorylase deficiency: autosomal recessive PYGM (11q13) disorder blocking muscle glycogen breakdown, causing exercise intolerance, second wind, rhabdomyolysis, and myoglobinuria; managed with carbohydrate-rich diet and supervised aerobic exercise.
Glycogen storage disease due to muscle phosphofructokinase deficiency
Tarui disease (垂井病), glycogen storage disease type VII, muscle phosphofructokinase deficiency: autosomal recessive PFKM defect blocking glycolysis, causing exercise intolerance, cramps, hemolysis, hyperuricemia; described 1965.
Glycogen storage disease due to muscle phosphorylase kinase deficiency
Glycogen storage disease due to muscle phosphorylase kinase deficiency (GSD IXα1, formerly GSD IXd), an ultra-rare X-linked PHKA1 disorder impairing muscle glycogen breakdown, causing mild exercise intolerance, myalgia, cramps, and weakness.
Glycogen storage disease due to phosphoglycerate mutase deficiency
Glycogen storage disease type X, also called GSD10 or muscle phosphoglycerate mutase deficiency, is a rare autosomal recessive myopathy causing exercise intolerance, cramps, and rhabdomyolysis, first described in 1981.
Glycogen storage disease type II
Glycogen storage disease type II, or Pompe disease, acid maltase deficiency, GAA deficiency: autosomal recessive lysosomal glycogen buildup from acid alpha-glucosidase deficiency, causing infantile cardiomyopathy or late-onset muscle weakness, treated with enzyme replacement therapy.
Glycogen storage disease with severe cardiomyopathy due to glycogenin deficiency
Glycogen storage disease type XV (GSD 15, GSDXV, OMIM 613507) is an ultra-rare autosomal recessive GYG1 (glycogenin-1) disorder causing severe polyglucosan cardiomyopathy, arrhythmias, and heart failure often requiring transplantation, with skeletal muscle usually spared.
Glycogen storage diseases
Glycogen storage disease (GSD), also called glycogenosis, is an inherited metabolic disorder in which defective enzymes prevent normal glycogen breakdown, affecting mainly the liver and muscles.
Glycogen synthase
Glycogen synthase (EC 2.4.1.11, GYS1/GYS2) is the rate-limiting UDP-glucose-dependent enzyme elongating glycogen's alpha-1,4 chains, regulated by phosphorylation and glucose-6-phosphate; GYS2 deficiency causes GSD type 0a.
Glycogen Synthase Kinase-3
Glycogen synthase kinase-3 (GSK-3, GSK3), serine/threonine kinase with paralogs GSK3α and GSK3β; phosphorylates primed substrates, regulates insulin and Wnt/β-catenin signaling; drug target for diabetes, Alzheimer's, and cancer.
Glycogenesis
Glycogenesis is the biosynthesis of glycogen from glucose via UDP-glucose, using glycogenin, glycogen synthase, and branching enzyme; insulin promotes it, glucagon and cortisol oppose it, and defects cause glycogen storage disease.
Glycogenin
Glycogenin (GYG1/GYG2): self-glucosylating glucosyltransferase priming glycogen synthesis by attaching glucose to Tyr194/195 via a glucose-tyrosine bond; later found dispensable in knockout mice.
Glycogenin-1
Glycogenin-1 (GYG1, 3q24) is the self-glucosylating primer enzyme of glycogen synthesis in human muscle and heart; biallelic GYG1 mutations cause glycogen storage disease XV with polyglucosan storage, and glycogen can form without it.