α-galactosidase a

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。

alpha-Galactosidase A 是一种溶酶体外糖苷酶，能够水解糖缀合物的末端 α 半乳糖部分。

α-Galactosidase(EC 3.2.1.22)，即 α-半乳糖苷酶，是一种糖苷水解酶，广泛存在于动植物和微生物中，常用于生化研究。α-Galactosidase 可以催化 α-1,6 连接的末端半乳糖残基的水解，包括低聚半乳糖、半乳甘露聚糖和半乳糖脂。催化许多分解代谢过程，包括糖蛋白、糖脂和多糖的裂解。

Raffinose (Melitriose) 是不能够消化的短链寡糖，是由半乳糖，葡萄糖和果糖组成的三糖，能够在许多植物中找到。它能够被 α-半乳糖苷酶 (α-GAL) 水解为 D-半乳糖和蔗糖。

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。

4-Nitrophenyl α-D-galactopyranoside (PNP-alpha-D-Gal) 是 4-硝基苯基 (pNP) 糖吡喃糖苷的人工底物，可用于 α-半乳糖苷酶活性的检测。它用作底物能够显著增加 pNP 的释放量。

X-alpha-Gal 是 α-半乳糖苷酶的底物。它用于区分酵母的α-半乳糖苷酶阳性菌株。

DGJ-pFPhT is a ligand of human α-galactosidase A.

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. Globotriaosylceramides act as natural resistance factors to HIV infection, interacting with HIV gp120 to prevent its interaction with chemokine co-receptors and subsequent fusion of HIV to host cell membranes. This product contains a mixture of hydroxy fatty acid-containing globotriaosylceramides isolated from porcine red blood cells (RBCs).

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. Globotriaosylceramides act as natural resistance factors to HIV infection, interacting with HIV gp120 to prevent its interaction with chemokine co-receptors and subsequent fusion of HIV to host cell membranes. This product contains a mixture of hydroxy and non-hydroxy fatty acid-containing globotriaosylceramides isolated from porcine red blood cells (RBCs).

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 disease, a lysosomal storage disorder characterized by a deficiency in the enzyme α-galactosidase A. It also accumulates in the urine, kidney, and plasma of patients with Fabry disease. Lyso-globotriaosylceramide levels decrease in response to administration of the α-galactosidase inhibitor 1-deoxygalactonojirimycin in a transgenic mouse model of Fabry disease. Decreases in plasma and urine concentrations of lyso-globotriaosylceramide have been used as a biomarker for efficacy of enzyme replacement therapy (ERT) and other therapies in the treatment of Fabry disease.

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 lysosomal storage disorder characterized by a deficiency in the enzyme α-galactosidase A. C18 globotriaosylceramide also accumulates in endothelial cells, pericytes, vascular smooth muscle cells, renal epithelial cells, dorsal ganglia neuronal cells, and myocardial cells in patients with Fabry disease.

Australine is a pyrrolizidine alkaloid originally isolated fromC. australethat has enzyme inhibitory activities.1,2,3It is an inhibitor of glucoamylase (IC50= 5.8 μM) that also inhibits glucosidase I, sucrase, maltase, andA. nigerα-glucosidase (IC50s = 20, 28, 35, and 28 μM, respectively).2,3Australine is selective for these enzymes over glucosidase II, α- and β-mannosidase, and α- and β-galactosidase up to 500 μM, β-glucosidase, with only 5% inhibition at 66 μM, as well as isomaltase and trehalase (IC50= 97 and 160 μM, respectively). Australine (500 μg/ml) inhibits glycoprotein processing of viral glycoproteins in influenza virus-infected MDCK cells and induces the accumulation of glycoproteins.2 1.Molyneux, R.J., Benson, M., Wong, R.Y., et al.Australine, a novel pyrrolizidine alkaloid glucosidase inhibitor from Castanospermum australJ. Nat. Prod.51(6)1198-1206(1988) 2.Tropea, J.E., Molyneux, R.J., Kaushal, G.P., et al.Australine, a pyrrolizidine alkaloid that inhibits amyloglucosidase and glycoprotein processingBiochemistry28(5)2027-2034(1989) 3.Kato, A., Kano, E., Adachi, I., et al.Australine and related alkaloids: easy structural confirmation by 13C NMR spectral data and biological activitiesTetrahedron Asymmetry14(3)325-331(2003)

Stachyose hydrate 是一种能够增强有益细菌的生长和活性的益生元。它具有降血糖作用，能够调节肠道微生物而改善炎症。

Migalastat (GR181413A free base) 是一种具有口服活性和竞争性的α-galactosidase A 抑制剂，对人α-Gal A 的IC50值为 0.04 μM。

4-Methylumbelliferyl 2-acetamido-2-deoxy-α-D-galactopyranoside是一种用于研究α-半乳糖苷酶活性的荧光底物。此化合物适用于分析各种生物样本中的酶活性，帮助科学家更深入地理解该酶在生物体内的角色。此外，4-Methylumbelliferyl 2-acetamido-2-deoxy-α-D-galactopyranoside还可以用于筛选出能够抑制或激活该酶的潜在化合物。

5-Bromo-4-chloro-1H-indol-3-yl nonanoate 是一种荧光底物，用于酶检测。它与多种酶反应并产生荧光产物，已被证实对 β-半乳糖苷酶、α-糜蛋白酶和 β-葡萄糖醛酸酶具有活性。