Recombinant Human GLalpha protein (His tag)

Abbreviation: GLα

Target Synonym: Alpha-Galactosidase A;Alpha-D-Galactosidase A;Alpha-D-Galactoside;Galactohydrolase;Melibiase;Agalsidase;GLA;GLAL

Target Species: Human

Expression Host: E.coli

Fusion Tag: N-His

UNIProt ID: P06280

Background: α-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. α-Galactosidase A can hydrolyze terminal α-galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose. Defects α-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.

Sequence: Leu 32-Leu 429

Purity: > 95 % as determined by reducing SDS-PAGE.

Formulation: Lyophilized from sterile PBS, pH 7.4.
Normally 5 % - 8 % trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization.
Please refer to the specific buffer information in the printed manual.

Endotoxin: Please contact us for more information.

• Artikelnummer: ELSPDEH100177-100
• Hersteller: Elabscience Biotechnology
• Hersteller Artikelnummer: ELA-PDEH100177-100
• Green Labware: Nein
• Verpackungseinheit: 100 µg
• Mengeneinheit: STK