Page last updated: 2024-10-06

Galactosemia II (GALK)

Proteins (8)

ProteinSynonymsTaxonomy
Hexokinase-4HK4; EC 2.7.1.1; Glucokinase; Hexokinase type IV; HK IV; Hexokinase-DHomo sapiens (human)
UDP-glucose 4-epimeraseEC 5.1.3.2; Galactowaldenase; UDP-N-acetylgalactosamine 4-epimerase; UDP-GalNAc 4-epimerase; UDP-N-acetylglucosamine 4-epimerase; UDP-GlcNAc 4-epimerase; 5.1.3.7; UDP-galactose 4-epimeraseHomo sapiens (human)
GalactokinaseEC 2.7.1.6; Galactose kinaseHomo sapiens (human)
Galactose-1-phosphate uridylyltransferaseGal-1-P uridylyltransferase; EC 2.7.7.12; UDP-glucose--hexose-1-phosphate uridylyltransferaseHomo sapiens (human)
UDP-glucose 6-dehydrogenaseUDP-Glc dehydrogenase; UDP-GlcDH; UDPGDH; EC 1.1.1.22Homo sapiens (human)
Phosphoglucomutase-1PGM 1; EC 5.4.2.2; Glucose phosphomutase 1Homo sapiens (human)
UTP--glucose-1-phosphate uridylyltransferaseEC 2.7.7.9; UDP-glucose pyrophosphorylase; UDPGP; UGPaseHomo sapiens (human)
UDP-glucuronic acid decarboxylase 1EC 4.1.1.35; UDP-glucuronate decarboxylase 1; UGD; UXS-1Homo sapiens (human)

Compounds (15)

CompoundDescription
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
NADH
galactoseAn aldohexose that occurs naturally in the D-form in lactose, cerebrosides, gangliosides, and mucoproteins. Deficiency of galactosyl-1-phosphate uridyltransferase (GALACTOSE-1-PHOSPHATE URIDYL-TRANSFERASE DEFICIENCY DISEASE) causes an error in galactose metabolism called GALACTOSEMIA, resulting in elevations of galactose in the blood.
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.
adenosine diphosphateAdenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.
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)
galactose-1-phosphate
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.
uridine diphosphate galactoseA nucleoside diphosphate sugar which can be epimerized into UDPglucose for entry into the mainstream of carbohydrate metabolism. Serves as a source of galactose in the synthesis of lipopolysaccharides, cerebrosides, and lactose.
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)
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.