GLA Protein (His tag)
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- Antigène Voir toutes GLA Protéines
- GLA (Galactosidase, alpha (GLA))
- Type de proteíne
- Recombinant
- Activité biologique
- Active
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Origine
- Souris
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Source
- HEK-293 Cells
- Purification/Conjugué
- Cette GLA protéine est marqué à la His tag.
- Fonction
- Recombinant Mouse alpha-Galactosidase A/GLA Protein (His Tag)(Active)
- Séquence
- Met1-Arg421
- Attributs du produit
- A DNA sequence encoding the mouse Gla (Q8BGZ6) (Met1-Arg421) was expressed with a C-terminal polyhistidine tag.
- Pureté
- > 95 % as determined by SDS-PAGE
- niveau d'endotoxine
- < 1.0 EU per μg of the protein as determined by the LAL method.
- Biological Activity Comment
- Measured by its ability to hydrolyze 4-methylumbelliferyl-α-D-galactopyranoside.The specific activity is > 400 pmoles/min/μg.
- Top Product
- Discover our top product GLA Protéine
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- Restrictions
- For Research Use only
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- Format
- Lyophilized
- Reconstitution
- Please refer to the printed manual for detailed information.
- Buffer
- Lyophilized from sterile PBS, pH 7.4
- Stock
- 4 °C,-20 °C,-80 °C
- Stockage commentaire
- 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.
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- Antigène
- GLA (Galactosidase, alpha (GLA))
- Autre désignation
- alpha-Galactosidase A/GLA (GLA Produits)
- Synonymes
- GALA Protein, Ags Protein, zgc:101584 Protein, MGC130872 Protein, SMU.877 Protein, SCF11.21 Protein, AO090005000217 Protein, alpha-GAL Protein, galactosidase alpha Protein, galactosidase, alpha Protein, galactosidase alpha S homeolog Protein, alpha-galactosidase Protein, aga Protein, alpha-galactosidase A Protein, GLA Protein, Gla Protein, gla Protein, gla.S Protein, agaN Protein, aga Protein, agaL Protein, SCO0541 Protein, rafA Protein, melA Protein, galA Protein, ANI_1_2528074 Protein, ANI_1_1502124 Protein, AOR_1_390174 Protein, CpipJ_CPIJ002066 Protein, MCYG_00962 Protein, MCYG_00791 Protein, Tsp_02909 Protein, Tsp_02508 Protein
- Sujet
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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.
Synonym: Ags
- Poids moléculaire
- 45.6 kDa
- UniProt
- Q8BGZ6
- Pathways
- SARS-CoV-2 Protein Interactome
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