Catalyzes the hydrolysisAstragaloside IVActivator proteinLipidNeuronsGlycosphingolipidsSubstratesEnzymeCatabolismMutationsProteinReactionProductComplexGlycosphingolipidsAccumulationBeta-galactosidaseLysosomesActivatorDegradationGlycoproteinsSubstrateGalactoseStimulateMembranesKineticsInfluenceCellsNormalBrainEffectStudiesStructureRemoval
Catalyzes the hydrolysis3
- This enzyme catalyzes the hydrolysis of a terminal beta-linked galactose residue from ganglioside substrates and other glycoconjugates. (thermofisher.com)
- β-galactosidase , also called lactase , beta-gal or β-gal , is a glycoside hydrolase enzyme that catalyzes the hydrolysis of β-galactosides into monosaccharides through the breaking of a glycosidic bond . (wikidoc.org)
- [10] The active site of β-galactosidase catalyzes the hydrolysis of its disaccharide substrate via "shallow" (nonproductive site) and "deep" (productive site) binding. (wikidoc.org)
Astragaloside IV3
- Cycloastragenol can also be derived from Astragaloside IV via hydrolysis action. (phcoker.com)
- While both cycloastragenol and astragaloside IV are derived from astragalus herb, cycloastragenol can also be obtained from astragaloside IV through hydrolysis process. (phcoker.com)
- Ang cycloastragenol ay maaari ding makuha mula sa Astragaloside IV sa pamamagitan ng aksyon na hydrolysis. (phcoker.com)
Activator protein2
- In the case of G M2 gangliosidosis, an activator protein is deficient, which leads to the accumulation of ganglioside G M2 and related glycolipids in neuronal cells. (medscape.com)
- The GM2A gene encodes the GM2 activator proteinM2 a binding protein required for the hydrolysis of GM2 gangliosides by the beta-hexosaminidase A. It forms a complex with GM2 gangliosideM2 which interacts specifically with beta-hexosaminidase A in the lysosomes (Gravel et al. (preventiongenetics.com)
Lipid2
- Gangliosides are active antioxidants, lipid peroxidation inhibitors. (doclecture.net)
- Lipidomics can reveal the mechanism of lipid oxidative hydrolysis at the level of lipid molecules, which will help to develop novel techniques to control the oxidative hydrolysis of lipids. (creative-proteomics.com)
Neurons2
- Then, as neurons become distended with GM2 gangliosides, a relentless deterioration of mental and physical abilities begins. (wikipedia.org)
- In adults, almost all of cerebrosides (90%) are in the myelin sheath, and gangliosides are in neurons. (doclecture.net)
Glycosphingolipids1
- Is key in the biosynthesis of glycosphingolipids and gangliosides. (foodb.ca)
Substrates1
- Substrates of different β-galactosidases include ganglioside GM1, lactosylceramides, lactose , and various glycoproteins . (wikidoc.org)
Enzyme2
- The mutation disrupts the activity of the enzyme, which results in the build-up of the molecule GM2 gangliosideM2within cells, leading to toxicity. (wikipedia.org)
- Ceramides (N-acylsphingosine) are one of the hydrolysis byproducts of sphingomyelin by the enzyme sphingomyelinase (sphingomyelin phosphorylcholine phosphohydrolase E.C.3.1.4.12) which has been identified in the subcellular fractions of human epidermis (PMID 25935) and many other tissues. (foodb.ca)
Catabolism1
- Normal products of all 3 genes are required for normal catabolism of the G M2 ganglioside substrate. (medscape.com)
Mutations1
- Crystallographic Structure of Human beta-Hexosaminidase A: Interpretation of Tay-Sachs Mutations and Loss of GM2 GangliosideM2Hydrolysis. (tu-muenchen.de)
Protein2
- 2015). Determining the composition of protein, carbohydrate, moisture, fat, and ash in raw EBN is essential for various purposes, including the hydrolysis process for the extraction of its valuable components. (researchgate.net)
- Serum paraoxonase, arylesterase and lactonase activities were determined by the measuring initial rate of substrate (paraoxon, phenylacetate and dihydrocoumarin) hydrolysis.Streptozotocin-induced diabetes mellitus caused a significant decrease whereas lipoic acid treatment caused a significant increase in serum PON1 and PON3 protein levels and paraoxonase, arylesterase and lactonase activities. (scite.ai)
Reaction1
- Various reactions occur in lipids during food processing, such as thermal oxidation, hydrolysis, thermal polymerization and Merad reaction. (creative-proteomics.com)
Product3
- Exposure of cells to either octanoic acid (C8:0), a C8-ceramide hydrolysis product, or oleic acid (C18:1) did not affect MDR1 expression. (sphingolipidclub.com)
- Hydrolyzed Fumonisin B2 (HFB2) is a hydrolysis product of fumonisins (HF), which retains biological activity. (medchemexpress.com)
- Hydrolyzed Fumonisin B1 (Aminopentol) is the backbone and main hydrolysis product of the mycotoxin Fumonisin B1 (HY-N6719). (medchemexpress.com)
Complex1
- Hydrolysis of G M2 ganglioside requires a normal G M2 ganglioside-G M2 activator-Hex A complex. (medscape.com)
Glycosphingolipids4
- 1. Accelerated transfer of neutral glycosphingolipids and ganglioside GM1 by a purified lipid transfer protein. (nih.gov)
- 2. Isolation of two glycolipid transfer proteins from bovine brain: reactivity toward gangliosides and neutral glycosphingolipids. (nih.gov)
- Is key in the biosynthesis of glycosphingolipids and gangliosides. (hmdb.ca)
- Thus, the transfer of a phosphorylcholine head group from phosphatidylcholine to ceramide yields another phospholipid, sphingomyelin (also sphingolipid) and the addition of carbohydrate groups from the sugar donor, UDP-hexose, yields complex glycosphingolipids (cerebrosides, sulfatides, gangliosides). (gerli.com)
Accumulation3
- In the case of GM2 gangliosidosis, an activator protein is deficient, which leads to the accumulation of ganglioside GM2 and related glycolipids in neuronal cells. (medscape.com)
- Clinical symptoms of progressive neurodegeneration and developmental delay are caused by accumulation of GM2 gangliosideM2in lysosomes, forming membranous cytoplasmic bodies (MCBs) in neuronal cells. (medscape.com)
- Impaired hydrolysis catalysed by ß-hexosaminidase A (HexA) leads to the accumulation of GM2 gangliosideM2in neuronal lysosomes. (bvsalud.org)
Beta-galactosidase1
- Saposin-B stimulates the hydrolysis of galacto-cerebroside sulfate by arylsulfatase A (EC 3.1.6.8), GM1 gangliosides by beta-galactosidase (EC 3.2.1.23) and globotriaosylceramide by alpha-galactosidase A (EC 3.2.1.22). (nih.gov)
Lysosomes1
- Within lysosomes, beta-hexosaminidase A forms part of a complex that breaks down a fatty substance called GM2 gangliosideM2found in cell membranes. (medlineplus.gov)
Activator5
- Hydrolysis of GM2 gangliosideM2requires a normal GM2 ganglioside-GM2 activator-Hex A complex. (medscape.com)
- 4. Activator protein required for the enzymatic hydrolysis of cerebroside sulfate. (nih.gov)
- Deficiency in urine of patients affected with cerebroside sulfatase activator deficiency and identity with activators for the enzymatic hydrolysis of GM1 gangliosideM1and globotriaosylceramide. (nih.gov)
- 5. A triple-binding-domain model explains the specificity of the interaction of a sphingolipid activator protein (SAP-1) with sulphatide, GM1-ganglioside and globotriaosylceramide. (nih.gov)
- 14. Identity of the activator proteins for the enzymatic hydrolysis of sulfatide, ganglioside GM1, and globotriaosylceramide. (nih.gov)
Degradation2
- Lysosomal beta-hexosaminidase A (Hex A) is essential for the degradation of GM2 gangliosides in the central and peripheral nervous system. (nih.gov)
- The influence of fly ash on hydrolysis, degradation and adsorption of methyl parathion in aqueous soil suspensions. (cdc.gov)
Glycoproteins1
- Isoform 1: Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans. (nih.gov)
Substrate1
- Normal products of all 3 genes are required for normal catabolism of the GM2 gangliosideM2substrate. (medscape.com)
Galactose2
- 6. Galactose oxidase action on GM1 gangliosideM1in micellar and vesicular dispersions. (nih.gov)
- Among the membrane gangliosides 1 to 5 that were able to react with galactose oxidase on B cells, ganglioside 3 was not detected in the mutant strain, and its absence was counterbalanced by the presence of a larger amount of ganglioside 1. (trnds.co)
Stimulate1
- Saposin-A and saposin-C stimulate the hydrolysis of glucosylceramide by beta-glucosylceramidase (EC 3.2.1.45) and galactosylceramide by beta-galactosylceramidase (EC 3.2.1.46). (nih.gov)
Membranes3
- 10. Kinetics of ganglioside transfer between liposomal and synaptosomal membranes. (nih.gov)
- 12. Interaction between glycophorin and ganglioside GM1 on liposomal membranes. (nih.gov)
- Kinetics of PTEN-mediated PI (3, 4, 5) P3 hydrolysis on solid supported membranes. (upenn.edu)
Kinetics1
- 16. Kinetics of transfer of gangliosides from their micelles to dipalmitoylphosphatidylcholine vesicles. (nih.gov)
Influence1
- 20. Influence of gangliosides GM1 and GD1a on structural and thermotropic properties of sonicated small 1,2-dipalmitoyl-L-alpha-phosphatidylcholine vesicles. (nih.gov)
Cells2
- Progressive damage caused by the buildup of GM2 gangliosideM2leads to the destruction of these cells, which causes the signs and symptoms of Tay-Sachs disease. (medlineplus.gov)
- AF102B and AF150(S) are partial agonists, but AF150, AF151, and AF151 (S) are full agonists in stimulating phosphoinositides hydrolysis or arachidonic acid release in these cells. (inrae.fr)
Normal1
Brain2
- 3. Evidence for the presence of a ganglioside transfer protein in brain. (nih.gov)
- Morphological changes observed in a somatic neurohybrid clonal cell line SB21B1 are accompanied by a 9 fold increase in the expression of mRNA sequences for tubulin 14 days following the addition of various brain gangliosides to the culture medium. (inrae.fr)
Effect1
- The addition of purified neuraminidase and various purified hexosaminidases exerted only a minimal synergistic effect on the hydrolysis of Tay-Sachs ganglioside in the lysosomal preparations from the control or patient with the O variant of Tay-Sachs disease. (eurekamag.com)
Studies1
- 19. Calorimetric studies on the interaction of gangliosides with phospholipids and myelin basic protein. (nih.gov)
Structure1
- Only the alpha-subunit active site can hydrolyze GM2 gangliosides due to a flexible loop structure that is removed post-translationally from beta, and to the presence of alphaAsn423 and alphaArg424. (nih.gov)
Removal1
- Upon removal of gangliosides, the expression of the tubulin message is restored to near basal levels. (inrae.fr)