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D-(+)-Melibiose monohydrate 99+%

Dodavatel: Thermo Scientific

Popis: CAS No.: 66009-10-7

Bezpečnostní list

D-(+)-Melibiose monohydrate, white powder

Dodavatel: MP Biomedicals

Popis: Melibiose is a reducing disaccharide formed by an alpha-1,6 linkage between galactose and glucose (D-Gal-α(1→6)-D-Glc). It can be formed by invertase mediated hydrolysis of raffinose, which produces melibiose and fructose. Melibiose can be broken down into its component saccharides, glucose and galactose, by the enzyme alpha-galactosidase.

α-D-(+)-Melibiose

Katalogové číslo: (J60439.06)

Dodavatel: Thermo Scientific

Popis: Appearance: White crystalline powder
Solubility: Soluble in water at 100mg/ml

Měrná jednotka: 1 * 5 g

Bezpečnostní list Certifikáty Akce

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Human recombinant alpha-Galactosidase A (from cells)

Katalogové číslo: (PRSI91-323)

Dodavatel: ProSci Inc.

Popis: alpha-Galactosidase A is a homodimeric glycoprotein that belongs to the glycosyl hydrolase 27 family. It is a lysosomal enzyme and used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. alpha-Galactosidase A can hydrolyze terminal alpha-galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose. Defects alpha-Galactosidase A are the cause of Fabry disease (FD) which is a rare X-linked sphingolipidosis disease with glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease.

Měrná jednotka: 1 * 50 µG

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