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Search Results for " fabry disease "

12

Compounds

Cat No. Product Name Synonyms Targets
T4473 Ibiglustat Venglustat,SAR402671,GZ402671 Transferase
Ibiglustat (GZ402671) is a potent and selective Glucosylceramide synthase inhibitor and ceramide glucosyltransferase inhibitor. Ibiglustat blocks the formation of glucosylceramide (GL-1), a key intermediate in the synthe...
T12038 Migalastat hydrochloride GR181413A hydrochloride,Migalastat HCl,GR181413A HCl Others
Migalastat hydrochloride (GR181413A) is an orally available, potent and competitive inhibitor of alpha-galactosidase A. It promotes the transport of alpha-galactosidase A to the lysosome and can be used in the study of F...
T37192 4-Methylumbelliferyl-α-D-Galactopyranoside 4MU-α-Gal Others
4-Methylumbelliferyl-α-D-Galactopyranoside (4MU-α-Gal) (4-MU-α-Gal) is a fluorogenic substrate of α-galactosidase. In addition to its use in characterizing novel α-galactosidases, 4-MU-α-Gal is used in assays to evaluate...
T32923 Lucerastat NBDGJ
Lucerastat is a very effective and selective α-gal A (α-D-galactosidase) inhibitor for the treatment of lipid storage diseases and Fabry disease.
T39105 Ibiglustat succinate GZ402671succinate,Venglustat succinate,Ibiglustat succinate,SAR402671succinate
Ibiglustat (Venglustat) succinate is an orally active, brain-penetrant inhibitor of glucosylceramide synthase (GCS). It is utilized in the investigation of Gaucher disease type 3, Parkinson's disease associated with GBA ...
T37438 C17 Globotriaosylceramide (d18:1/17:0) C17 Globotriaosylceramide (d18:1/17:0)
C17 Globotriaosylceramide is a sphingolipid that has been used as an internal standard for the quantification of globotriaosylceramides in plasma and urine from patients with Fabry disease by GC-MS.
T37291 Lyso-Globotriaosylceramide (d18:1) Lyso-Globotriaosylceramide (d18:1)
Lyso-globotriaosylceramide is a form of globotriaosylceramide that is lacking the fatty acyl group. It binds to Shiga toxin 1 (Stx1) in the presence of cholesterol and phosphatidylcholine but does not bind Stx2. It also ...
T37439 C18 Globotriaosylceramide (d18:1/18:0)
C18 globotriaosylceramide is an endogenous sphingolipid found in mammalian cell membranes that is synthesized from lactosylceramide . It inhibits aggregation of human neutrophils induced by phorbol 12-myristate 13-acetat...
T37440 C2 Adamantanyl Galactosylceramide (d18:1/2:0)
C2 Adamantanyl Galactosylceramide (d18:1/2:0) (AdaGalCer) is a bioactive sphingolipid. It reduces globotriaosylceramide 3 synthesis from exogenous lactosylceramide in microsomes. AdaGalCer stimulates recombinant glucocer...
T36859 C16 Globotriaosylceramide (d18:1/16:0) C16 Globotriaosylceramide (d18:1/16:0)
C16 globotriaosylceramide is an endogenous sphingolipid found in mammalian cell membranes that is synthesized from C16 lactosylceramide . C16 globotriaosylceramide acts as a receptor for Shiga toxin in B cell-derived Raj...
T36185 Globotriaosylceramides (hydroxy) (porcine)
Globotriaosycleramides are glycosphingolipids found in mammalian cell membranes that are synthesized from lactosylceramides . They act as receptors for Shiga and Shiga-like toxins in vitro and in vivo. Globotriaosylceram...
T36186 Globotriaosylceramides (porcine)
Globotriaosycleramides are glycosphingolipids found in mammalian cell membranes that are synthesized from lactosylceramides . They act as receptors for Shiga and Shiga-like toxins in vitro and in vivo. Globotriaosylceram...
TargetMol