Page last updated: 2024-10-06

Glycogen Synthetase Deficiency

Proteins (16)

ProteinSynonymsTaxonomy
UDP-glucuronosyltransferase 2B11UDPGT 2B11; EC 2.4.1.17Homo sapiens (human)
Hexokinase-4HK4; EC 2.7.1.1; Glucokinase; Hexokinase type IV; HK IV; Hexokinase-DHomo sapiens (human)
Glycogen debranching enzymeGlycogen debrancherHomo sapiens (human)
Glucose-6-phosphate isomeraseGPI; EC 5.3.1.9; Autocrine motility factor; AMF; Neuroleukin; NLK; Phosphoglucose isomerase; PGI; Phosphohexose isomerase; PHI; Sperm antigen 36; SA-36Homo sapiens (human)
Maltase-glucoamylase, intestinalHomo sapiens (human)
Sucrase-isomaltase, intestinalHomo sapiens (human)
Glycogen [starch] synthase, liverEC 2.4.1.11Homo sapiens (human)
Glycogen phosphorylase, liver formEC 2.4.1.1Homo sapiens (human)
Hexokinase-2EC 2.7.1.1; Hexokinase type II; HK II; Hexokinase-B; Muscle form hexokinaseHomo sapiens (human)
Beta-glucuronidaseEC 3.2.1.31; Beta-G1Homo sapiens (human)
UDP-glucose 6-dehydrogenaseUDP-Glc dehydrogenase; UDP-GlcDH; UDPGDH; EC 1.1.1.22Homo sapiens (human)
Glucose 1,6-bisphosphate synthaseEC 2.7.1.106; PMMLP; Phosphoglucomutase-2-like 1Homo sapiens (human)
Phosphoglucomutase-1PGM 1; EC 5.4.2.2; Glucose phosphomutase 1Homo sapiens (human)
1,4-alpha-glucan-branching enzymeEC 2.4.1.18; Brancher enzyme; Glycogen-branching enzymeHomo sapiens (human)
UTP--glucose-1-phosphate uridylyltransferaseEC 2.7.7.9; UDP-glucose pyrophosphorylase; UDPGP; UGPaseHomo sapiens (human)
RecName: Full=Alpha-amylase 1A; AltName: Full=1,4-alpha-D-glucan glucanohydrolase 1; AltName: Full=Salivary alpha-amylase; Flags: PrecursorHomo sapiens (human)

Compounds (21)

CompoundDescription
Orthophosphate
NADH
Adenosine TriphosphateAn adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.
sucroseA nonreducing disaccharide composed of GLUCOSE and FRUCTOSE linked via their anomeric carbons. It is obtained commercially from SUGARCANE, sugar beet (BETA VULGARIS), and other plants and used extensively as a food and a sweetener.
adenosine diphosphateAdenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.
uridine diphosphateA uracil nucleotide containing a pyrophosphate group esterified to C5 of the sugar moiety.
uridine triphosphateUridine 5'-(tetrahydrogen triphosphate). A uracil nucleotide containing three phosphate groups esterified to the sugar moiety.
WaterA clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
glycogen
caloreenglucose polymer with average length of five glucose units for dietary energy supplement
acebutololA cardioselective beta-1 adrenergic antagonist with little effect on the bronchial receptors. The drug has stabilizing and quinidine-like effects on cardiac rhythm, as well as weak inherent sympathomimetic action.
isovalerylglucuronidemetabolite in urine of patients with isovaleric acidemia
uridine diphosphate glucuronic acidA nucleoside diphosphate sugar which serves as a source of glucuronic acid for polysaccharide biosynthesis. It may also be epimerized to UDP iduronic acid, which donates iduronic acid to polysaccharides. In animals, UDP glucuronic acid is used for formation of many glucosiduronides with various aglycones.
glucose-1-phosphateRN given refers to (alpha-D-Glc)-isomer
NADA coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
Glucose-6-PhosphateAn 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)
glycerate 1,3-biphosphate
3-phosphoglycerate
glucose-1,6-bisphosphate
pyrophosphate
glucoseA 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.