The biosynthesis of monoglycosylceramides requires a direct transfer of the carbohydrate moiety from a sugar-nucleotide, such as uridine 5-diphosphate(UDP)-galactose, or UDP-glucose to the ceramide unit. (wikipedia.org)
MS = An enzyme that catalyzes the transfer of UMP from UDPglucose to galactose 1-phosphate, forming UDPgalactose and glucose 1-phosphate. (doctorinternet.com)
Mannose and galactose content increased as the temperature is raised to 37°C whereas glucose and fucose content decreased. (biotechjournal.in)
The addition of a phosphocholine group to ceramide yields sphingomyelin, but the addition of glucose or galactose to ceramide yields glycosphingolipids and sulfatides. (frontiersin.org)
Hydroxyl group of ceramide1
Cerebrosides which contain as their polar head group a galactose moiety bound in glycosidic linkage to the hydroxyl group of ceramide. (uchicago.edu)
Sphingolipids1
GALC degrades galactosylceramide, a major component of myelin, and other terminal beta-galactose-containing sphingolipids, including psychosine (galactosylsphingosine). (medscape.com)
Sulfatides3
Cerebrosides containing a sulfuric ester (sulfate) group, known as sulfatides, also occur in the myelin sheath of nerves. (wikipedia.org)
A nucleoside diphosphate sugar which serves as a source of N-acetylgalactosamine for glycoproteins , sulfatides and cerebrosides. (lookformedical.com)
In addition, ceramide can be converted to sulfatides by the action of galactosylceramide synthase followed by cerebroside sulfotransferase (CST). (frontiersin.org)
Sphingosine1
Cerebroside molecules are able form up to eight intermolecular hydrogen bonds between the polar hydrogens of the sugar and the hydroxy and amide groups of the sphingosine base of the ceramide. (wikipedia.org)
Uridyltransferase1
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. (lookformedical.com)
Monoglycosyl and oligoglycosylceramides having a mono or polysaccharide bonded glycosidically to the terminal OH group of ceramide are defined as cerebrosides. (wikipedia.org)
Group1
Cerebrosides is the common name for a group of glycosphingolipids called monoglycosylceramides which are important components in animal muscle and nerve cell membranes. (wikipedia.org)
Glycosphingolipids4
Cerebrosides is the common name for a group of glycosphingolipids called monoglycosylceramides which are important components in animal muscle and nerve cell membranes. (wikipedia.org)
Fabry's disease is a rare, X-linked disorder of the glycosphingolipid metabolism, in which a partial or total deficiency of a lysosomal alpha(α)-galactosidase results in the progressive accumulation of neutral glycosphingolipids with terminal alpha galactose moieties (i.e., cerebroside di- and trihexoside) in most body fluids and tissues. (ewha.ac.kr)
Lactose is a readily available source of galactose, which is essential to the production of the galactolipids, including cerebroside. (chototvinhlong.com)
Prompt removal of galactose/lactose from their diet resolves or prevents these acute symptoms. (nih.gov)
The common acute and potentially lethal symptoms of generalized epimerase deficiency galactosemia are prevented or corrected by a galactose/lactose-restricted diet. (nih.gov)
Note: Affected individuals may require trace environmental sources of galactose: infants should be fed a formula (e.g., soy formula) that contains trace levels of galactose or lactose. (nih.gov)
infants with intermediate epimerase deficiency galactosemia may benefit in the long term from early dietary galactose/lactose restriction, but this remains unclear. (nih.gov)
In generalized epimerase deficiency galactosemia, restriction of dietary galactose/lactose appears to correct or prevent the common acute signs and symptoms of the disorder (hepatic dysfunction, renal dysfunction, and mild cataracts), but not the developmental delay or learning impairment observed in some affected individuals. (nih.gov)
Glycolipids3
The melting point of cerebrosides is considerably greater than physiological body temperature, >37.0 °C, giving glycolipids a paracrystalline, similar to liquid crystal structure. (wikipedia.org)
Galactose is also synthesized in the body and forms part of glycolipids, used to maintain the cell membrane's stability and facilitate cellular recognition, and glycoproteins, which serve many bodily functions, including immunity. (biopureus.com)
Glycolipids can be simple, such as cerebroside, containing only one sugar molecule, or more complex. (profound-answers.com)
Myelin4
Cerebroside synthesis can therefore give a measurement of myelin formation or remyelination. (wikipedia.org)
Cerebrosides containing a sulfuric ester (sulfate) group, known as sulfatides, also occur in the myelin sheath of nerves. (wikipedia.org)
4. Reversal of non-hydroxy:alpha-hydroxy galactosylceramide ratio and unstable myelin in transgenic mice overexpressing UDP-galactose:ceramide galactosyltransferase. (nih.gov)
GALC degrades galactosylceramide, a major component of myelin, and other terminal beta-galactose-containing sphingolipids, including psychosine (galactosylsphingosine). (medscape.com)
Ceramide7
The fundamental structure of a cerebroside is ceramide. (wikipedia.org)
Monoglycosyl and oligoglycosylceramides having a mono or polysaccharide bonded glycosidically to the terminal OH group of ceramide are defined as cerebrosides. (wikipedia.org)
Cerebroside molecules are able form up to eight intermolecular hydrogen bonds between the polar hydrogens of the sugar and the hydroxy and amide groups of the sphingosine base of the ceramide. (wikipedia.org)
3. Modest phenotypic improvements in ASA-deficient mice with only one UDP-galactose:ceramide-galactosyltransferase gene. (nih.gov)
5. UDP-galactose:ceramide galactosyltransferase is a class I integral membrane protein of the endoplasmic reticulum. (nih.gov)
10. Association of the Golgi UDP-galactose transporter with UDP-galactose:ceramide galactosyltransferase allows UDP-galactose import in the endoplasmic reticulum. (nih.gov)
Sugars1
Galactose is one of eight essential sugars needed in the diet for proper cell development and functioning of the human body. (biopureus.com)
Sugar2
Galactose is an essential sugar utilized in cellar development and function. (biopureus.com)
Galactose is a naturally occurring versatile, simple sugar (monosaccharide) used in glycolysis to generate energy in the form of ATP. (biopureus.com)
Component1
Galactose is a crucial component of cerebroside, which can aid in an infant's brain growth. (farmdidi.com)