GLA/alpha-Galactosidase A Protein, Mouse, Recombinant (His)

GLA/alpha-Galactosidase A Protein, Mouse, Recombinant (His) is expressed in HEK293 mammalian cells with His tag. The predicted molecular weight is 45.6 kDa and the accession number is Q8BGZ6.

GLA/alpha-Galactosidase A Protein, Mouse, Recombinant (His)

パッケージサイズ	在庫状況	単価(税別)
50 μg	約5 days	￥ 161,000

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説明	GLA/alpha-Galactosidase A Protein, Mouse, Recombinant (His) is expressed in HEK293 mammalian cells with His tag. The predicted molecular weight is 45.6 kDa and the accession number is Q8BGZ6.

Species	Mouse
Expression Host	HEK293 Cells
Tag	C-His
Accession Number	Q8BGZ6
別名	galactosidase, alpha, Ags, galactosidase, α, GLA/α-Galactosidase A Protein
Construction	Mouse Gla (Q8BGZ6) (Met1-Arg421)
Protein Purity	> 95 % as determined by SDS-PAGE
分子量	45.6 kDa (predicted)

Endotoxin	< 1.0 EU/μg of the protein as determined by the LAL method.
Formulation	Lyophilized from a solution filtered through a 0.22 μm filter, containing PBS, pH 7.4. Typically, a mixture containing 5% to 8% trehalose, mannitol, and 0.01% Tween 80 is incorporated as a protective agent before lyophilization.
Reconstitution	A Certificate of Analysis (CoA) containing reconstitution instructions is included with the products. Please refer to the CoA for detailed information.
Stability & Storage	It is recommended to store recombinant proteins at -20°C to -80°C for future use. Lyophilized powders can be stably stored for over 12 months, while liquid products can be stored for 6-12 months at-80℃. For reconstituted proteinsolutions, the solution can be stored at -20°c to -80'c for at least 3 months. Please avoid multiple freeze-thaw cycles and store products in aliquots.
Shipping	In general, Lyophilized powders are shipping with blue ice.
Research Background	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.

参考文献

1. Koide T.et al., 1990, FEBS Lett. 259:353-356. 2. Yang C.-C. et al., 2003, Clin. Genet. 63:205-209. 3. Verovnik F. et al.,2004, Eur. J. Hum. Genet. 12:678-681. 4. Nance C.S. et al., 2006, Arch. Neurol. 63:453-457.

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GLA/alpha-Galactosidase A Protein, Mouse, Recombinant (His)

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