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抑制剂 & 化合物

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Cat. No.	Product Name	Target	Signaling Pathways
T4473	Ibiglustat Venglustat,SAR402671,GZ402671	Transferase	Metabolism
	Ibiglustat (GZ402671) 是一种可透过血脑屏障的、具有口服活性的葡萄糖神经酰胺合成酶(GCS)抑制剂。它可用于戈谢病 3 型、法布瑞氏症、与 GBA 突变相关的帕金森病、GM2 神经节苷脂病和常染色体显性多囊肾病的研究。
T12038	Migalastat hydrochloride GR181413A hydrochloride,Migalastat HCl,GR181413A HCl	Others	Others
	Migalastat hydrochloride (GR181413A) 是一种可口服且具有有效和竞争性的 α-galactosidase A 抑制剂，可促进α-半乳糖苷酶A向溶酶体的运输，可用于研究法布里病。
T37192	4-Methylumbelliferyl-α-D-Galactopyranoside 4MU-α-Gal	Others	Others
	4-Methylumbelliferyl-α-D-Galactopyranoside (4MU-α-Gal) (4-MU-α-Gal)是α-半乳糖苷酶的荧光底物。除了用于表征新的α-半乳糖苷酶外，4-MU-α-Gal 还用于评估与法布里病相关的α-半乳糖糖苷酶活性的缺乏。
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 mutations, Fabry disease, GM2 gangliosidosis, and autosomal dominant polycystic kidney disease.
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.
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-acetate (PMA; 10008014) when used at a concentration of 1 μM. C18 globotriaosylceramide acts as a receptor for Shiga toxin in B cell-derived Raji cells and THP-1 monocytes. It accumulates in the brain, heart, kidney, liver, lung, and spleen in a mouse model of Fabry disease, a lysosoma...
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 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.
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 Raji cells and THP-1 monocytes. It accumulates in endothelial cells, pericytes, vascular smooth muscle cells, renal epithelial cells, dorsal ganglia neuronal cells, and myocardial cells in patients with Fabry disease. C16 globotriaosylceramide is also upregulated in plasma of patien...
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. Globotriaosylceramides accumulate in endothelial cells, pericytes, vascular smooth muscle cells, renal epithelial cells, dorsal ganglia neuronal cells, and myocardial cells in patients with Fabry disease, a lysosomal storage disorder characterized by a deficiency in the enzyme α-galactosidase A. G...
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. Globotriaosylceramides accumulate in endothelial cells, pericytes, vascular smooth muscle cells, renal epithelial cells, dorsal ganglia neuronal cells, and myocardial cells in patients with Fabry disease, a lysosomal storage disorder characterized by a deficiency in the enzyme α-galactosidase A. G...

化合物

Ibiglustat

Cat.No: T4473

Synonym: Venglustat,SAR402671,GZ402671

Target: Transferase

Migalastat hydrochloride

Cat.No: T12038

Synonym: GR181413A hydrochloride,Migalastat HCl,GR181413A HCl

Target: Others

4-Methylumbelliferyl-α-D-Galactopyranoside

Cat.No: T37192

Synonym: 4MU-α-Gal

Synonym: GZ402671succinate,Venglustat succinate,Ibiglustat succinate,SAR402671succinate

Target:

C17 Globotriaosylceramide (d18:1/17:0)

Cat.No: T37438

Synonym: C17 Globotriaosylceramide (d18:1/17:0)

Target:

C18 Globotriaosylceramide (d18:1/18:0)

Cat.No: T37439

Synonym:

Target:

C2 Adamantanyl Galactosylceramide (d18:1/2:0)

Cat.No: T37440

Synonym:

Target:

C16 Globotriaosylceramide (d18:1/16:0)

Cat.No: T36859

Synonym: C16 Globotriaosylceramide (d18:1/16:0)

Target:

Globotriaosylceramides (hydroxy) (porcine)

Cat.No: T36185

Synonym:

Target:

Globotriaosylceramides (porcine)

Cat.No: T36186

Synonym:

Target:

Cat. No.	Product Name	Target	Signaling Pathways
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 reduces viability and aggregation of human neutrophils induced by phorbol 12-myristate 13-acetate when used at concentrations of 50 and 1 μM, respectively. Lyso-globotriaosylceramide accumulates in the brain, heart, kidney, liver, lung, and spleen in a mouse model of Fabry diseas...

天然产物

Lyso-Globotriaosylceramide (d18:1)

Cat.No: T37291

Synonym: Lyso-Globotriaosylceramide (d18:1)

Target:

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