Alpha-galactosidase A, also known as Alpha-D-galactoside galactohydrolase, Alpha-D-galactosidase A, Melibiase and GLA, is a member of the glycosyl hydrolase 27 family. GLA is used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. Defects in GLA are the cause of Fabry disease (FD) which is a rare X-linked sphingolipidosis disease where 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. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. 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. Deficiency of GLA leads to the accumulation of glycosphingolipids in the vasculature leading to multiorgan pathology. In addition to well-described microvascular disease, deficiency of GLA is also characterized by premature macrovascular events such as stroke and possibly myocardial infarction.
Tag
His tag
Description
A DNA sequence encoding the mouse Gla (Q8BGZ6) (Met1-Arg421) was expressed with a C-terminal polyhistidine tag.
Regulatory
RUO
Host
Human Cells
Endotoxin Level
< 1.0 EU per μg of the protein as determined by the LAL method
Nature
Recombinant
Shipping
In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature.Bulk packages of recombinant proteins are provided as frozen liquid. They are shipped out with blue ice unless customers require otherwise.
Buffer
Lyophilized from sterile PBS, pH 7.4
Description
Please refer to the printed manual for detailed information.
Purity
> 95 % as determined by SDS-PAGE
Expiry Date
Samples are stable for up to twelve months from date of receipt at -70℃
Immunogen Species
Mus Musculus (Mouse)
Attachment
Reviews of Recombinant Mouse alpha-Galactosidase A/GLA Protein (His Tag)(Active)