Recombinant Human GLα protein (His tag)

Specifications

Catalog number

PDEH100833-01

Size / count

20 µg

Dry ice

No

Hazardous

No

Discontinued

No

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.

Abbreviation

GLα

Synonyms

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

UniProt

P06280

Accession Number

P06280

Expression System

E.coli

Tag

N-His

Sequence

Leu 32-Leu 429

Field of Research

Cardiovascular

Endotoxin

< 10 EU/mg of the protein as determined by the LAL method.

Purity

> 95% as determined by reducing SDS-PAGE.

Bioactivity

Not validated for activity

Reconstitution

It is recommended that sterile water be added to the vial to prepare a stock solution of 0.5 mg/mL. Concentration is measured by UV-Vis.

Shipping Conditions

This product is provided as lyophilized powder which is shipped with ice packs.

Storage Conditions

Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months.

Calculated Molecular Weight

43.7 kDa

Observed Molecular Weight

48 kDa

Species

Human