For research use only. Not for therapeutic Use.
Trihexosylceramide (d18:1/12:0) is a type of Trihexosylceramide. In the renal tissue of heterozygous Fabry’s disease, the cytoplasm of glomerular epithelial cells appears foamy. Trihexosylceramide accumulated in glomerular epithelial cells as identified by immunostaining[1][2].
| Catalog Number | I041248 |
| CAS Number | 474943-80-1 |
| Synonyms | N-[(E,2S,3R)-1-[(2R,3R,4R,5S,6R)-3,4-dihydroxy-6-(hydroxymethyl)-5-[(2S,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxyoxan-2-yl]oxy-3-hydroxyoctadec-4-en-2-yl]dodecanamide |
| Molecular Formula | C42H79NO13 |
| Purity | ≥95% |
| InChI | InChI=1S/C42H79NO13/c1-3-5-7-9-11-13-14-15-16-18-19-21-23-25-31(46)30(43-34(47)26-24-22-20-17-12-10-8-6-4-2)29-53-41-39(52)37(50)40(33(28-45)55-41)56-42-38(51)36(49)35(48)32(27-44)54-42/h23,25,30-33,35-42,44-46,48-52H,3-22,24,26-29H2,1-2H3,(H,43,47)/b25-23+/t30-,31+,32+,33+,35-,36-,37+,38+,39+,40+,41+,42-/m0/s1 |
| InChIKey | KNWHKVBHCLQVFX-CZUYTOTJSA-N |
| SMILES | CCCCCCCCCCCCCC=CC(C(COC1C(C(C(C(O1)CO)OC2C(C(C(C(O2)CO)O)O)O)O)O)NC(=O)CCCCCCCCCCC)O |
| Reference | [1]. Wang JR, et al. Improved sphingolipidomic approach based on ultra-high performance liquid chromatography and multiple mass spectrometries with application to cellular neurotoxicity. Anal Chem. 2014 Jun 17;86(12):5688-96. [2]. Fukushima M, et al. A female heterozygous patient with Fabry’s disease with renal accumulation of trihexosylceramide detected with a monoclonal antibody. Am J Kidney Dis. 1995 Dec;26(6):952-5. |
