N-Acetylmannosamine
When the GNE epimerase kinase does not function correctly in the human body thereby reducing the available ManNAc, it is ... terminal monosaccharides of carbohydrate chains that are attached to glycoproteins and glycolipids (glycans). ManNAc is the ... In the rate-limiting step of the pathway, UDP-GlcNAc is converted into ManNAc by UDP-GlcNAc 2-epimerase, encoded by the ... The UDP-GlcNAc 2-epimerase kinase is the rate limiting step in sialic acid biosynthesis. If the enzyme does not work ...
Nucleotide sugar
"The Development of Carbohydrate Chemistry and Biology". Carbohydrate Chemistry, Biology and Medical Applications: 1-28. doi: ... is a congenital disease resulted from altered function of UDP-GlcNAc epimerase . Macular corneal dystrophy: is a congenital ... Carbohydrate chemistry EamA Glycorandomization Glycosyltransferase Nucleotide sugars metabolism Derek Horton (2008). " ...
Isomerase
The sub-categories of isomerases containing racemases, epimerases and cis-trans isomers are examples of enzymes catalyzing the ... Isomerases catalyze reactions across many biological processes, such as in glycolysis and carbohydrate metabolism. Examples of ... Racemases act upon molecules with one chiral carbon for inversion of stereochemistry, whereas epimerases target molecules with ... and epimerases). These isomerases invert stereochemistry at the target chiral carbon. ...
Beta oxidation
Many ruminant animals form a large amount of 3-carbon propionate during the fermentation of carbohydrates in the rumen. Long- ... However, the D conformation is enzymatically converted into the L conformation by methylmalonyl-CoA epimerase, then it ... but instead use carbohydrates (red blood cells and neurons) or ketone bodies (neurons only). Because many fatty acids are not ...
Galactosemia
... and epimerase deficiency with glucose.[citation needed] Accumulated galactose can also undergo an alternative reaction: ... the accumulation of galactose becomes the substrate for enzymes that catalyze the polyol pathway of carbohydrate metabolism. ... Dysmetria Diminished bone density Premature ovarian failure Cataract Galactosemic cataract Other Inborn errors of carbohydrate ...
Psicose
... but requires its weight listing as a carbohydrate, with 0.4 kcal/g (about 1/10 the calories of ordinary carbohydrates). A meta- ... Fructose can be converted to allulose by the enzyme D-tagatose 3-epimerase, which has allowed for mass production of allulose. ... The caloric value of allulose in humans is about 0.2 to 0.4 kcal/g, relative to about 4 kcal/g for typical carbohydrates. In ... Overall, compared to the carbohydrate-containing meal alone, the same meal with a small dose of added allulose resulted in a 10 ...
Heparan sulfate
Epimerisation is catalysed by one enzyme, the GlcA C5 epimerase or heparosan-N-sulfate-glucuronate 5-epimerase (EC 5.1.3.17). ... Heparan sulfate is a member of the glycosaminoglycan family of carbohydrates and is very closely related in structure to ... 2-O-sulfotransferase and C5-epimerases". Developmental Dynamics. 236 (2): 581-6. doi:10.1002/dvdy.21051. PMID 17195182. S2CID ... These enzymes consist of multiple glycosyltransferases, sulfotransferases and an epimerase. These same enzymes also synthesize ...
Calvin cycle
The carbohydrate products of the Calvin cycle are three-carbon sugar phosphate molecules, or "triose phosphates", namely, ... Xu5P is converted into RuP by phosphopentose epimerase. Finally, phosphoribulokinase (another plant-unique enzyme of the ... Surplus G3P can also be used to form other carbohydrates such as starch, sucrose, and cellulose, depending on what the plant ...
Galactose-1-phosphate uridylyltransferase deficiency
UDP-galactose can then be converted to lactose, by the enzyme lactose synthase or to UDP-glucose by UDP-galactose epimerase ( ... Inborn errors of carbohydrate metabolism, Autosomal recessive disorders). ... epimerase (GALE)". Molecular Genetics and Metabolism. 93 (2): 160-71. doi:10.1016/j.ymgme.2007.09.012. PMC 2253667. PMID ... including galactokinase deficiency and galactose epimerase deficiency. Further analysis of metabolites and enzyme activities ...
Galactose
... epimerase (GALE).[citation needed] In human lactation, galactose is required in a 1 to 1 ratio with glucose to enable the ... a class of natural polymeric carbohydrates. D-Galactose is also known as brain sugar since it is a component of glycoproteins ( ... epimerase (GALE) interconverts UDP-galactose and UDP-glucose, thereby completing the pathway. The above mechanisms for ...
Galactogen
Goudsmit EM (1972). "Carbohydrates and carbohydrate metabolism in Mollusca.". In Florkin M, Scheer BT (eds.). Chemical Zoology ... Both use glucose as a common precursor and its conversion to activated galactose is catalyzed by UDP-glucose 4-epimerase and ... Given that carbohydrates retain water, the high amount of this polysaccharide would protect the eggs from desiccation from ... Carbohydrate Research. 44 (2): 241-249. doi:10.1016/S0008-6215(00)84167-1. ISSN 0008-6215. Holst O, Mayer H, Okotore RO, König ...
Galactosemic cataract
Infant formula based on casein hydrolysates and dextrin maltose as a carbohydrate source can also be used for initial ... Online Mendelian Inheritance in Man (OMIM): Galactose Epimerase Deficiency - 230350 Shin YS, Korenke GC, Huppke P, Knerr I, ... epimerase (GALE), is the cause of Type III galactosemia. GALE deficiency is an extremely rare, autosomal recessive disease that ... which catalyzes the next step in the carbohydrate metabolic cycle. Thus, the sugar alcohol idly begins to accumulate in the ...
List of enzymes
EC 5.1.3 UDP-glucose 4-epimerase Category:EC 5.1.99 Methylmalonyl CoA epimerase Category:EC 5.2 FKBP: FKBP1A FKBP1B FKBP2 FKBP3 ... and complex carbohydrates to be turned into simple sugars that will be easier to absorb. Clinical Significance: Amylase also ...
N-acetyl-D-glucosamine kinase
"Use of a cell-free system to determine UDP-N-acetylglucosamine 2-epimerase and N-acetylmannosamine kinase activities in human ... a major component of complex carbohydrates, from lysosomal degradation or nutritional sources into GlcNAc 6-phosphate. NAGK ...
O-GlcNAc
As UDP-GlcNAc is the final product of the hexosamine biosynthetic pathway, which integrates amino acid, carbohydrate, fatty ... epimerase (GALE). Treatment with Ac4GalNAz was found to result in enhanced labeling of O-GlcNAc relative to Ac4GlcNAz, possibly ... This pathway integrates information about the concentrations of various metabolites including amino acids, carbohydrates, fatty ... CS1: long volume value, Post-translational modification, Carbohydrates, Biochemistry, Cell signaling, Cell biology, Signal ...
Post-translational modification
Many eukaryotic and prokaryotic proteins also have carbohydrate molecules attached to them in a process called glycosylation, ... via the cyclisation of asparagine or aspartic acid amino-acid residues racemization of serine by protein-serine epimerase of ...
List of OMIM disorder codes
CPS1 Carbohydrate-deficient glycoprotein syndrome, type Ib; 602579; MPI Carboxypeptidase N deficiency; 212070; CPN1 Carcinoid ... MMAB Methylmalonyl-CoA epimerase deficiency; 251120; MCEE Mevalonic aciduria; 610377; MVK MHC class II deficiency, ... GALK1 Galactose epimerase deficiency; 230350; GALE Galactosemia; 230400; GALT Galactosialidosis; 256540; CTSA Gallbladder ...