126550-86-5 , Globotriaosylsphingosine , Lysoglobotriaosylceramide; Ceramide trihexoside; lysoGb3

126550-86-5 , Globotriaosylsphingosine ,
Lysoglobotriaosylceramide;
Ceramide trihexoside;
lysoGb3 ,
a-D-Gal-(1→4)-b-D-Gal-(1→4)-b-D-Glc-1→O-sphingosine;
Cas:126550-86-5
C36H67NO17 / 785.91
MFCD00675433

Globotriaosylsphingosine ,

Lysoglobotriaosylceramide;

Ceramide trihexoside; lysoGb3 ,
a-D-Gal-(1→4)-b-D-Gal-(1→4)-b-D-Glc-1→O-sphingosine;

Lysogb3 is a type of glycosphingolipid, which is a membrane lipid that contains a sphingoid base and one or more sugar residues. It is found in the plasma membrane of human and animal cells, where it plays a crucial role in cellular signaling, cell-cell recognition, and immune responses. Lysogb3 is synthesized from its precursor molecule, globotriaosylceramide (Gb3), by the enzymatic action of a specific hydrolase, called lysosomal beta-galactosidase. Defects in this enzyme lead to the accumulation of Gb3 and its derivatives, causing severe lysosomal storage disorders, such as Fabry disease.

CAS Number	126550-86-5
Product Name	Lysogb3
IUPAC Name	(2R,3R,4S,5R,6R)-2-[(2R,3R,4R,5R,6S)-6-[(2R,3S,4R,5R,6R)-6-[(E,2S,3R)-2-amino-3-hydroxyoctadec-4-enoxy]-4,5-dihydroxy-2-(hydroxymethyl)oxan-3-yl]oxy-4,5-dihydroxy-2-(hydroxymethyl)oxan-3-yl]oxy-6-(hydroxymethyl)oxane-3,4,5-triol
Molecular Formula	C₃₆H₆₇NO₁₇
Molecular Weight	785.91 g/mol
InChI	InChI=1S/C36H67NO17/c1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-21(41)20(37)19-49-34-30(47)27(44)32(23(17-39)51-34)54-36-31(48)28(45)33(24(18-40)52-36)53-35-29(46)26(43)25(42)22(16-38)50-35/h14-15,20-36,38-48H,2-13,16-19,37H2,1H3/b15-14+/t20-,21+,22+,23+,24+,25-,26-,27+,28+,29+,30+,31+,32+,33-,34+,35+,36-/m0/s1
InChI Key	GRGNVOCPFLXGDQ-TWHXEDJUSA-N
SMILES	CCCCCCCCCCCCCC=CC(C(COC1C(C(C(C(O1)CO)OC2C(C(C(C(O2)CO)OC3C(C(C(C(O3)CO)O)O)O)O)O)O)O)N)O
Synonyms	((2S,3R,4E)-2-Amino-3-hydroxy-4-octadecenyl O-α-D-galactopyranosyl-(1→4)-O-β-D-galactopyranosyl-(1→4)-β-D-glucopyranoside; lyso-Gb3; (2R,3R,4S,5R,6R)-2-(((2R,3R,4R,5R,6S)-6-(((2R,3S,4R,5R,6R)-6-(((2S,3R,E)-2-Amino-3-hydroxyoctadec-4-en-1-yl)oxy)-4,5
Canonical SMILES	CCCCCCCCCCCCCC=CC(C(COC1C(C(C(C(O1)CO)OC2C(C(C(C(O2)CO)OC3C(C(C(C(O3)CO)O)O)O)O)O)O)O)N)O
Isomeric SMILES	CCCCCCCCCCCCC/C=C/[C@H]([C@H](CO[C@H]1[C@@H]([C@H]([C@@H]([C@H](O1)CO)O[C@H]2[C@@H]([C@H]([C@H]([C@H](O2)CO)O[C@@H]3[C@@H]([C@H]([C@H]([C@H](O3)CO)O)O)O)O)O)O)O)N)O