Reaction Process: WikiPathways:WP5173
Disorders of galactose metabolism related metabolites
find 6 related metabolites which is associated with chemical reaction(pathway) Disorders of galactose metabolism
galactose ⟶ Galactitol
Galactitol
Galactitol or dulcitol is a sugar alcohol that is a metabolic breakdown product of galactose. Galactose is derived from lactose in food (such as dairy products). When lactose is broken down by the enzyme lactase it produces glucose and galactose. Galactitol has a slightly sweet taste. It is produced from galactose in a reaction catalyzed by aldose reductase. When present in sufficiently high levels, galactitol can act as a metabotoxin, a neurotoxin, and a hepatotoxin. A neurotoxin is a compound that disrupts or attacks neural cells and neural tissue. A hepatotoxin as a compound that disrupts or attacks liver tissue or liver cells. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of galactitol are associated with at least two inborn errors of metabolism, including galactosemia and galactosemia type II. Galactosemia is a rare genetic metabolic disorder that affects an individuals ability to metabolize the sugar galactose properly. Excess lactose consumption in individuals with galactose intolerance or galactosemia activates aldose reductase to produce galactitol, thus depleting NADPH and leading to lowered glutathione reductase activity. As a result, hydrogen peroxide or other free radicals accumulate causing serious oxidative damage to various cells and tissues. In individuals with galactosemia, the enzymes needed for the further metabolism of galactose (galactose-1-phosphate uridyltransferase) are severely diminished or missing entirely, leading to toxic levels of galactose 1-phosphate, galactitol, and galactonate. High levels of galactitol in infants are specifically associated with hepatomegaly (an enlarged liver), cirrhosis, renal failure, cataracts, vomiting, seizure, hypoglycemia, lethargy, brain damage, and ovarian failure. Galactitol is an optically inactive hexitol having meso-configuration. It has a role as a metabolite, a human metabolite, an Escherichia coli metabolite and a mouse metabolite. Galactitol is a metabolite found in or produced by Escherichia coli (strain K12, MG1655). Galactitol is a natural product found in Elaeodendron croceum, Salacia chinensis, and other organisms with data available. Galactitol is a naturally occurring product of plants obtained following reduction of galactose. It appears as a white crystalline powder with a slight sweet taste. It may form in excess in the lens of the eye in galactosemias a deficiency of galactokinase. A naturally occurring product of plants obtained following reduction of GALACTOSE. It appears as a white crystalline powder with a slight sweet taste. It may form in excess in the lens of the eye in GALACTOSEMIAS, a deficiency of GALACTOKINASE. A naturally occurring product of plants obtained following reduction of galactose. It appears as a white crystalline powder with a slight sweet taste.; Dulcitol (or galactitol) is a sugar alcohol, the reduction product of galactose. Galactitol in the urine is a biomarker for the consumption of milk. Galactitol is found in many foods, some of which are elliotts blueberry, italian sweet red pepper, catjang pea, and green bean. An optically inactive hexitol having meso-configuration. COVID info from COVID-19 Disease Map Corona-virus Coronavirus SARS-CoV-2 COVID-19 SARS-CoV COVID19 SARS2 SARS Acquisition and generation of the data is financially supported in part by CREST/JST. Dulcite is a sugar alcohol with a slightly sweet taste which is a metabolic breakdown product of galactose. Dulcite is a sugar alcohol with a slightly sweet taste which is a metabolic breakdown product of galactose.
Glucose 6-phosphate
Glucose 6 phosphate (alpha-D-glucose 6 phosphate or G6P) is the alpha-anomer of glucose-6-phosphate. There are two anomers of glucose 6 phosphate, the alpha anomer and the beta anomer. Glucose 6 phosphate is 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). Glucose-6-phosphate is a phosphorylated glucose molecule on carbon 6. When glucose enters a cell, it is immediately phosphorylated to G6P. This is catalyzed with hexokinase enzymes, thus consuming one ATP. A major reason for immediate phosphorylation of the glucose is so that it cannot diffuse out of the cell. The phosphorylation adds a charged group so the G6P cannot easily cross cell membranes. G6P can travel down two metabolic pathways, glycolysis and the pentose phosphate pathway. In addition to the metabolic pathways, G6P can also be stored as glycogen in the liver if blood glucose levels are high. If the body needs energy or carbon skeletons for syntheses, G6P can be isomerized to Fructose-6-phosphate and then phosphorylated to Fructose-1,6-bisphosphate. Note, the molecule now has 2 phosphoryl groups attached. The addition of the 2nd phosphoryl group is an irreversible step, so once this happens G6P will enter glycolysis and be turned into pyruvate (ATP production occurs). If blood glucose levels are high, the body needs a way to store the excess glucose. After being converted to G6P, phosphoglucose mutase (isomerase) can turn the molecule into glucose-1-phosphate. Glucose-1-phosphate can then be combined with uridine triphosphate (UTP) to form UDP-glucose. This reaction is driven by the hydrolysis of pyrophosphate that is released in the reaction. Now, the activated UDP-glucose can add to a growing glycogen molecule with the help of glycogen synthase. This is a very efficient storage mechanism for glucose since it costs the body only 1 ATP to store the 1 glucose molecule and virtually no energy to remove it from storage. It is important to note that glucose-6-phosphate is an allosteric activator of glycogen synthase, which makes sense because when the level of glucose is high the body should store the excess glucose as glycogen. On the other hand, glycogen synthase is inhibited when it is phosphorylated by protein kinase a during times of high stress or low blood glucose levels. -- Wikipedia [HMDB] Glucose 6-phosphate (G6P, sometimes called the Robison ester) is a glucose sugar phosphorylated at the hydroxy group on carbon 6. Glucose 6-phosphate (G6P) has two anomers: the alpha anomer and the beta anomer. Glucose 6-phosphate is 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). When glucose enters a cell, it is immediately phosphorylated to G6P. This is catalyzed with hexokinase enzymes, thus consuming one ATP. A major reason for immediate phosphorylation of the glucose is so that it cannot diffuse out of the cell. The phosphorylation adds a charged group so the G6P cannot easily cross cell membranes. G6P can travel down two metabolic pathways: glycolysis and the pentose phosphate pathway. In addition to the metabolic pathways, G6P can also be stored as glycogen in the liver if blood glucose levels are high. If the body needs energy or carbon skeletons for syntheses, G6P can be isomerized to fructose 6-phosphate and then phosphorylated to fructose 1,6-bisphosphate. Note, the molecule now has 2 phosphoryl groups attached. The addition of the 2nd phosphoryl group is an irreversible step, so once this happens G6P will enter glycolysis and be turned into pyruvate (ATP production occurs). If blood glucose levels are high, the body needs a way to store the excess glucose. After being converted to G6P, phosphoglucose mutase (an isomerase) can turn the molecule into glucose 1-phosphate. Glucose 1-phosphate can then be combined with uridine triphosphate (UTP) to form UDP-glucose. This reaction is driven by the hydrolysis of pyrophosphate that is released in the reaction. Now, the activated UDP-glucose can add to a growing glycogen molecule with the help of glycogen synthase. This is a very efficient storage mechanism for glucose since it costs the body only 1 ATP to store the 1 glucose molecule and virtually no energy to remove it from storage. It is important to note that glucose 6-phosphate is an allosteric activator of glycogen synthase, which makes sense because when the level of glucose is high the body should store the excess glucose as glycogen. On the other hand, glycogen synthase is inhibited when it is phosphorylated by protein kinase during times of high stress or low blood glucose levels. Acquisition and generation of the data is financially supported in part by CREST/JST. CONFIDENCE standard compound; INTERNAL_ID 237 KEIO_ID G003; [MS2] KO009109 KEIO_ID G003
α-D-Glucose-1-phosphate
Glucose 1-phosphate (also called cori ester) is a glucose molecule with a phosphate group on the 1-carbon. It can exist in either the α- or β-anomeric form. Glucose 1-phosphate belongs to the class of organic compounds known as monosaccharide phosphates. These are monosaccharides comprising a phosphated group linked to the carbohydrate unit. Glucose 1-phosphate is the direct product of the reaction in which glycogen phosphorylase cleaves off a molecule of glucose from a greater glycogen structure. It cannot travel down many metabolic pathways and must be interconverted by the enzyme phosphoglucomutase in order to become glucose 6-phosphate. Free glucose 1-phosphate can also react with UTP to form UDP-glucose. It can then return to the greater glycogen structure via glycogen synthase. *Found widely in both plants and animals. A precursor of starch in plants and of glycogen in animals. [CCD] Acquisition and generation of the data is financially supported in part by CREST/JST. COVID info from COVID-19 Disease Map KEIO_ID G020 Corona-virus KEIO_ID G115 Coronavirus SARS-CoV-2 COVID-19 SARS-CoV COVID19 SARS2 SARS
Uridine diphosphategalactose
Uridine diphosphategalactose (UDPgal) is a nucleoside diphosphate sugar which can be epimerized into UDPglucose for entry into the mainstream of carbohydrate metabolism. UDPgal is a pivotal compound in the metabolism of galactose. UDPgal is a product of the galactose-l-phosphate uridyl transferase (EC 2.7.7.10) reaction but may also be made from Glucose-l-P, involving uridine diphosphate galactose-4-epimerase (EC 5.1.3.2). UDPgal is the necessary galactosyl donor of galactose in the metabolism to incorporate it into complex oligosaccharides, glycoproteins and glycolipids (galactosides). Defective galactosylation of complex glycoconjugates exists in tissues from galactosemic patients. There is a tendency for galactosemic red cell UDPgal to be in the low normal range with a high uridine diphosphate glucose to UDP-gal ratio. This may reflect an inability of red cell UDPgal-4-epimerase to maintain a normal ratio and consequently higher levels of UDPgal. In the more complex white blood cells and cultured fibroblasts, the UDPgal content and the uridine diphosphate glucose to UDPgal ratio of galactosemics are normal. Therefore, defective galactosylation observed in galactosemic fibroblasts must result from a defect in the transfer of galactose from UDPgal to these moieties. (PMID: 2122114, 7671968) [HMDB] Uridine diphosphategalactose (UDPgal) is a nucleoside diphosphate sugar which can be epimerized into UDPglucose for entry into the mainstream of carbohydrate metabolism. UDPgal is a pivotal compound in the metabolism of galactose. UDPgal is a product of the galactose-l-phosphate uridyl transferase (EC 2.7.7.10) reaction but may also be made from Glucose-l-P, involving uridine diphosphate galactose-4-epimerase (EC 5.1.3.2). UDPgal is the necessary galactosyl donor of galactose in the metabolism to incorporate it into complex oligosaccharides, glycoproteins and glycolipids (galactosides). Defective galactosylation of complex glycoconjugates exists in tissues from galactosemic patients. There is a tendency for galactosemic red cell UDPgal to be in the low normal range with a high uridine diphosphate glucose to UDP-gal ratio. This may reflect an inability of red cell UDPgal-4-epimerase to maintain a normal ratio and consequently higher levels of UDPgal. In the more complex white blood cells and cultured fibroblasts, the UDPgal content and the uridine diphosphate glucose to UDPgal ratio of galactosemics are normal. Therefore, defective galactosylation observed in galactosemic fibroblasts must result from a defect in the transfer of galactose from UDPgal to these moieties. (PMID: 2122114, 7671968). Acquisition and generation of the data is financially supported in part by CREST/JST. COVID info from COVID-19 Disease Map Corona-virus Coronavirus SARS-CoV-2 COVID-19 SARS-CoV COVID19 SARS2 SARS
Galactose 1-phosphate
Galactose 1-phosphate, also known as D-Galactose-1-phosphate or alpha-D-gal-1-P, belongs to the class of organic compounds known as monosaccharide phosphates. These are monosaccharides comprising a phosphate group linked to the carbohydrate unit. Galactose-1-phosphate is an intermediate in the interconversion of glucose and uridine diphosphate galactose. Galactose 1-phosphate exists in all living species, ranging from bacteria to plants to humans. Within humans, galactose 1-phosphate participates in a number of enzymatic reactions. In particular, uridine diphosphate glucose and galactose 1-phosphate can be biosynthesized from uridine diphosphategalactose and glucose 1-phosphate; which is mediated by the enzyme galactose-1-phosphate uridylyltransferase (GALT). In addition, galactose 1-phosphate can be biosynthesized from D-galactose through the action of the enzyme galactokinase. The improper metabolism of galactose-1-phosphate is a characteristic of a condition known as galactosemia (PMID: 7671964). Type I galactosemia is a genetic disorder that is caused by the impairment of galactose-1-phosphate uridylyltransferase (EC 2.7.7.12). Evidence suggests that misfolding of the galactose 1-phosphate uridylyltransferase enzyme is the underlying cause of type I galactosemia (PMID: 23583749). Outside of the human body, galactose 1-phosphate has been detected, but not quantified in, several different foods, such as gooseberries, anises, turmerics, caraway, and cumins. COVID info from COVID-19 Disease Map Occurs in liver, milk, and yeasts Corona-virus Coronavirus SARS-CoV-2 COVID-19 SARS-CoV COVID19 SARS2 SARS Acquisition and generation of the data is financially supported in part by CREST/JST.