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GCK | glucokinase (hexokinase 4); Catalyzes the initial step in utilization of glucose by the beta-cell and liver at physiological glucose concentration. Glucokinase has a high Km for glucose, and so it is effective only when glucose is abundant. The role of GCK is to provide G6P for the synthesis of glycogen. Pancreatic glucokinase plays an important role in modulating insulin secretion. Hepatic glucokinase helps to facilitate the uptake and conversion of glucose by acting as an insulin-sensitive determinant of hepatic glucose usage (466 aa) | |||
A4GALT | alpha 1,4-galactosyltransferase; Necessary for the biosynthesis of the Pk antigen of blood histogroup P. Catalyzes the transfer of galactose to lactosylceramide and galactosylceramide. Necessary for the synthesis of the receptor for bacterial verotoxins (353 aa) | |||
G6PC | glucose-6-phosphatase, catalytic subunit; Hydrolyzes glucose-6-phosphate to glucose in the endoplasmic reticulum. Forms with the glucose-6-phosphate transporter (SLC37A4/G6PT) the complex responsible for glucose production through glycogenolysis and gluconeogenesis. Hence, it is the key enzyme in homeostatic regulation of blood glucose levels (357 aa) | |||
GNS | glucosamine (N-acetyl)-6-sulfatase (552 aa) | |||
HEXB | hexosaminidase B (beta polypeptide); Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues (556 aa) | |||
HEXA | hexosaminidase A (alpha polypeptide); Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. The form B is active against certain oligosaccharides. The form S has no measurable activity (529 aa) | |||
GALNS | galactosamine (N-acetyl)-6-sulfate sulfatase (522 aa) | |||
G6PC3 | glucose 6 phosphatase, catalytic, 3; Hydrolyzes glucose-6-phosphate to glucose in the endoplasmic reticulum. May form with the glucose-6-phosphate transporter (SLC37A4/G6PT) a ubiquitously expressed complex responsible for glucose production through glycogenolysis and gluconeogenesis. Probably required for normal neutrophil function (346 aa) | |||
GALM | galactose mutarotase (aldose 1-epimerase); Mutarotase converts alpha-aldose to the beta-anomer. It is active on D-glucose, L-arabinose, D-xylose, D-galactose, maltose and lactose (By similarity) (342 aa) | |||
AKR1B1 | aldo-keto reductase family 1, member B1 (aldose reductase); Catalyzes the NADPH-dependent reduction of a wide variety of carbonyl-containing compounds to their corresponding alcohols with a broad range of catalytic efficiencies (316 aa) | |||
HK2 | hexokinase 2 (917 aa) | |||
HK3 | hexokinase 3 (white cell) (923 aa) | |||
LALBA | lactalbumin, alpha-; Regulatory subunit of lactose synthase, changes the substrate specificity of galactosyltransferase in the mammary gland making glucose a good acceptor substrate for this enzyme. This enables LS to synthesize lactose, the major carbohydrate component of milk. In other tissues, galactosyltransferase transfers galactose onto the N-acetylglucosamine of the oligosaccharide chains in glycoproteins (142 aa) | |||
GUSB | glucuronidase, beta; Plays an important role in the degradation of dermatan and keratan sulfates (651 aa) | |||
GLB1 | galactosidase, beta 1; Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans (677 aa) | |||
B4GALT2 | UDP-Gal-betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2 (401 aa) | |||
ST3GAL1 | ST3 beta-galactoside alpha-2,3-sialyltransferase 1; It may be responsible for the synthesis of the sequence NeuAc-alpha-2,3-Gal-beta-1,3-GalNAc- found on sugar chains O- linked to Thr or Ser and also as a terminal sequence on certain gangliosides. SIAT4A and SIAT4B sialylate the same acceptor substrates but exhibit different Km values (340 aa) | |||
B4GALNT1 | beta-1,4-N-acetyl-galactosaminyl transferase 1; Involved in the biosynthesis of gangliosides GM2, GD2 and GA2 (533 aa) | |||
ST3GAL2 | ST3 beta-galactoside alpha-2,3-sialyltransferase 2; It may be responsible for the synthesis of the sequence NeuAc-alpha-2,3-Gal-beta-1,3-GalNAc- found in terminal carbohydrate groups of certain glycoproteins, oligosaccharides and glycolipids. SIAT4A and SIAT4B sialylate the same acceptor substrates but exhibit different Km values (350 aa) | |||
HKDC1 | hexokinase domain containing 1 (917 aa) | |||
GM2A | GM2 ganglioside activator; The large binding pocket can accommodate several single chain phospholipids and fatty acids, GM2A also exhibits some calcium-independent phospholipase activity (By similarity). Binds gangliosides and stimulates ganglioside GM2 degradation. It stimulates only the breakdown of ganglioside GM2 and glycolipid GA2 by beta-hexosaminidase A. It extracts single GM2 molecules from membranes and presents them in soluble form to beta- hexosaminidase A for cleavage of N-acetyl-D-galactosamine and conversion to GM3 (193 aa) | |||
AKR1B10 | aldo-keto reductase family 1, member B10 (aldose reductase); Acts as all-trans-retinaldehyde reductase. Can efficiently reduce aliphatic and aromatic aldehydes, and is less active on hexoses (in vitro). May be responsible for detoxification of reactive aldehydes in the digested food before the nutrients are passed on to other organs (316 aa) | |||
UGCG | UDP-glucose ceramide glucosyltransferase; Catalyzes the first glycosylation step in glycosphingolipid biosynthesis, the transfer of glucose to ceramide. May also serve as a "flippase" (394 aa) | |||
GBA2 | glucosidase, beta (bile acid) 2; Non-lysosomal glucosylceramidase that catalyzes the conversion of glucosylceramide (GlcCer) to free glucose and ceramide. Involved in sphingomyelin generation and prevention of glycolipid accumulation. May also catalyze the hydrolysis of bile acid 3-O-glucosides, however, the relevance of such activity is unclear in vivo (927 aa) | |||
B4GALT1 | UDP-Gal-betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1 (398 aa) | |||
HK1 | hexokinase 1 (921 aa) |