Recombinant Human Alpha-Galactosidase A/GLA Protein (His Tag)

SKU: PKSH033249

Size: 50μg

Storage: Store at < -20°C, stable for 6 months. Please minimize freeze-thaw cycles.

Shipping: This product is provided as liquid. It is shipped at frozen temperature with blue ice/gel packs. Upon receipt, store it immediately at < - 20°C.

Exp date: 12 months

Category ID_II: Recombinant Proteins

Category ID_III: Others

Abbreviation: Alpha-Galactosidase A;GLA

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

Research Areas: Cardiovascular

Conjugation:

Target Species: Human

Expression Host: HEK293 Cells

Application:

Fusion tag: C-His

UNIProt ID: P06280

Accession: 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.

Concentration:

Activity: Not validated for activity

Sequence: Leu32-Leu429

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

Formulation: Supplied as a 0.2 μm filtered solution of 20mM Tris-HCl, 150mM NaCl, pH 8.0.

Reconstitution: Not Applicable

Endotoxin: < 1.0 EU per μg of the protein as determined by the LAL method.

Calculated MW: 46.4 kDa

ObservedMW: 50-60 kDa