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| Product | Description | |
|---|---|---|
| Globotriaosylceramide | Globotriaosylceramide is a synthetic compound utilized for studying Anderson-Fabry disease. Acting as a substrate for enzymes, it aids in the research of this lysosomal storage disorder. Synonyms: Gb3; a-D-Gal-(1?4)-b-D-Gal-(1?4)-b-D-Glc-1?O-ceramide; Ceramide trihexosides. CAS No. 71965-57-6. Molecular formula: C60H113NO18. Mole weight: 1136.54. |  |
| globotriaosylceramide 3-β-N-acetylgalactosaminyltransferase | Globoside is a neutral glycosphingolipid in human erythrocytes and has blood-group-P-antigen activity. The enzyme requires a divalent cation for activity, with Mn2+ required for maximal activity. UDP-GalNAc is the only sugar donor that is used efficiently by the enzyme: UDP-Gal and UDP-GlcNAc result in very low enzyme activity. Lactosylceramide, globoside and gangliosides GM3 and GD3 are not substrates. For explanation of the superscripted '3' in the systematic name, see GL-5.3.4. Group: Enzymes. Synonyms: uridine diphosphoacetylgalactosamine-galactosylgalactosylglucosylceramide acetylgalactosaminyltransferase; globoside synthetase; UDP-N-acetylgalactosamine: globotriaosylceramide β-3-N-acetylgalactosaminyltransferase; galactosylgalactosylglucosylceramide β-D-acetylgalactosaminyltransferase; UDP-N-acetylgalactosamine: globotriaosylceramide β1,3-N-acetylgalactosaminyltrans. Enzyme Commission Number: EC 2.4.1.79. CAS No. 62213-46-1. Storage: Store it at +4 ?C for short term. For long term storage, store it at -20 ?C?-80 ?C. Form: Liquid or lyophilized powder. EXWM-2619; globotriaosylceramide 3-β-N-acetylgalactosaminyltransferase; EC 2.4.1.79; 62213-46-1; uridine diphosphoacetylgalactosamine-galactosylgalactosylglucosylceramide acetylgalactosaminyltransferase; globoside synthetase; UDP-N-acetylgala |  |
| 2,5-Dideoxy-2,5-imino-D-mannitol HCl | 2,5-Dideoxy-2,5-imino-D-mannitol HCl, a highly efficacious pharmaceutical compound, occupies a prominent position in the biomedical landscape owing to its profound therapeutic value in tackling Fabry disease. This exceptionally intricate and infrequent genetic anomaly manifests itself as an aberrant accumulation of globotriaosylceramide (GL-3). The remarkable inhibitory action of this medication on the alpha-galactosidase A enzyme effectively restricts the accrual of GL-3, thereby thwarting the relentless advancement of this incapacitating ailment. Synonyms: 2,5-Anhydro-2,5-imino-D-mannitol HCl. CAS No. 210115-92-7. Molecular formula: C6H13NO4.HCl. Mole weight: 199.63. | |
| α-1,4-Galactosyltransferase from Neisseria meningitides, Recombinant | Lactosylceramide 4-alpha-galactosyltransferase is an enzyme that in humans is encoded by the A4GALT gene. The protein encoded by this gene catalyzes the transfer of galactose to lactosylceramide to form globotriaosylceramide, which has been identified as the P(k) antigen of the P blood group system. The encoded protein, which is a type II membrane protein found in the Golgi, is also required for the synthesis of the bacterial verotoxins receptor. Group: Enzymes. Synonyms: lactosylceramide 4-alpha-galactosyltransferase; Galbeta1-4Glcbeta1-Cer alpha1,4-galactosyltransferase; globotriaosylceramide/CD77 synthase; histo-blood group Pk UDP-ga. Enzyme Commission Number: EC 2.4.1.228. CAS No. 52725-57-2. Purity: min 95% by SDS-PAGE. Galactosyltransferase. Mole weight: 34 kDa. Source: E. coli. Species: Neisseria meningitides. lactosylceramide 4-alpha-galactosyltransferase; Galbeta1-4Glcbeta1-Cer alpha1,4-galactosyltransferase; globotriaosylceramide/CD77 synthase; histo-blood group Pk UDP-galactose; UDP-galactose:lactosylceramide 4II-alpha-D-galactosyltransferase; UDP-galactose:beta-D-galactosyl-(1->4)-D-glucosyl(1<->1)ceramide 4II-alpha-D-galactosyltransferase; UDP-galactose:beta-D-galactosyl-(1->4)-D-glucosyl-(1<->1)-ceramide 4II-alpha-D-galactosyltransferase; α-1,4-Galactosyltransferase. Cat No: NATE-1479. | |
| C2 Adamantanyl Galactosylceramide (d18:1/2:0) | C2 Adamantanyl Galactosylceramide (d18:1/2:0) is a bioactive sphingolipid. It reduces globotriaosylceramide 3 synthesis from exogenous lactosylceramide in microsomes. AdaGalCer stimulates recombinant glucocerebrosidase activity in a pH-dependent manner. It activates glucocerebrosidase to decrease glucosylceramide accumulation in fibroblasts and lymphoblasts isolated from patients with Gaucher and Fabry disease, respectively. Synonyms: [(2S,3R,4E)-2-[(1-Adamantyl)acetylamino]-3-hydroxy-4-octadecenyl]beta-D-galactopyranoside; N-(1-Adamantaneacetyl)-galactosylceramide; Adamantanyl Galactosyl(β) Ceramide; AdaGalCer(d18:1/2:0). Grades: ≥98%. CAS No. 574738-16-2. Molecular formula: C36H63NO8. Mole weight: 637.89. | |
| Globotriose | Globotriose is a carbohydrate product used in biomedicine to study the role of glycolipids in various diseases and drug development. It is commonly utilized as a mimic to study the biological interactions with globotriaosylceramide (Gb3), which is involved in the progression of certain infections, such as E. coli and Shiga toxin-producing bacteria. Globotriose provides valuable insights into the development of therapeutics targeting Gb3-mediated diseases. Synonyms: 4-O-(4-O-α-D-Galactopyranosyl-β-D-galactopyranosyl)-D-glucopyranose; Gal-a-1,4-Gal-b-1,4-Glc; pk-Antigen; Globotriaose; O-α-D-Galactopyranosyl-(1?4)-O-β-D-galactopyranosyl-(1?4)-D-glucose; alpha-D-Gal-(1->4)-beta-D-Gal-(1->4)-D-Glc; alpha-D-Galp-(1->4)-beta-D-Galp-(1->4)-D-Glcp; Globotriose. Grades: ≥95%. CAS No. 66580-68-5. Molecular formula: C18H32O16. Mole weight: 504.44. | |
| lactosylceramide 4-α-galactosyltransferase | For explanation of superscript II in systematic name, see 2-carb.37. Group: Enzymes. Synonyms: Galβ1-4Glcβ1-Cer α1,4-galactosyltransferase; globotriaosylceramide/CD77 synthase; histo-blood group Pk UDP-galactose; UDP-galactose:lactosylceramide 4II-α-D-galactosyltransferase; UDP-galactose:β-D-galactosyl-(1?4)-D-glucosyl(1<->1)ceramide 4II-α-D-galactosyltransferase; UDP-galactose:β-D-galactosyl-(1?4)-D-glucosyl-(1<->1)-ceramide 4II-α-D-galactosyltransferase. Enzyme Commission Number: EC 2.4.1.228. CAS No. 52725-57-2. Galactosyltransferase. Storage: Store it at +4 ?C for short term. For long term storage, store it at -20 ?C?-80 ?C. Form: Liquid or lyophilized powder. EXWM-2457; lactosylceramide 4-α-galactosyltransferase; EC 2.4.1.228; 52725-57-2; Galβ1-4Glcβ1-Cer α1,4-galactosyltransferase; globotriaosylceramide/CD77 synthase; histo-blood group Pk UDP-galactose; UDP-galactose:lactosylceramide 4II-α-D-galactosyltransferase; UDP-galactose:β-D-galactosyl-(1?4)-D-glucosyl(1<->1)ceramide 4II-α-D-galactosyltransferase; UDP-galactose:β-D-galactosyl-(1?4)-D-glucosyl-(1<->1)-ceramide 4II-α-D-galactosyltransferase. Cat No: EXWM-2457. | |
| Migalastat HCl | Migalastat HCl, also known as AT1001 or GR181413A, is a pharmacological chaperone that selectively binds, stabilizes, and increases cellular levels of α-Gal A. Oral administration of migalastat HCl reduces tissue GL-3 in Fabry transgenic mice, and in urine and kidneys of some FD patients. Migalastat HCl may provide a potential novel genotype-specific treatment for Fabry Disease (FD). Phase 3 studies are ongoing. Fabry disease (FD) is a genetic disorder resulting from deficiency of the lysosomal enzyme α-galactosidase A (α-Gal A) which leads to globotriaosylceramide (GL-3) accumulation in multiple tissues. Synonyms: 1,5-Dideoxy-1,5-imino-D-galactitol HCl; DGJ; (2R,3S,4R,5S)-2-methylolpiperidine-3,4,5-triol hydrochloride; Migalastat hydrochloride; Amigal. Grades: 98%. CAS No. 75172-81-5. Molecular formula: C6H13NO4.HCl. Mole weight: 199.63. | |
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