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

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GLA / alpha-Galactosidase A Protein, Human, Recombinant (His Tag): Product Information

Purity
> 97 % as determined by SDS-PAGE
Endotoxin
< 1.0 EU per μg of the protein as determined by the LAL method
Activity
Testing in progress
Protein Construction
A DNA sequence encoding the human GLA (NP_000160.1) (Met 1-Leu 429) was fused with a polyhistidine tag at the C-terminus.
Accession#
Expressed Host
HEK293 Cells
Species
Human
Predicted N Terminal
Leu 32
Molecule Mass
The secreted recombinant human GLA consists of 409 amino acids and has a predicted molecular mass of 46.8 kDa. In SDS-PAGE under reducing conditions, it migrates as an approximately 50 kDa band.
Formulation
Lyophilized from sterile 50mM Tris, 150mM NaCl, pH 7.5
1. Normally 5 % - 8 % trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization. Specific concentrations are included in the hardcopy of COA.
2. Please contact us for any concerns or special requirements.
Please refer to the specific buffer information in the hard copy of CoA.
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.
Stability & Storage
Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃
Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
Reconstitution
A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.

GLA / alpha-Galactosidase A Protein, Human, Recombinant (His Tag): Images

GLA / alpha-Galactosidase A Protein, Human, Recombinant (His Tag): Alternative Names

GALA Protein, Human

GLA / alpha-Galactosidase A Background Information

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.
Full Name
galactosidase, alpha
References
  • Koide T.et al., 1990, FEBS Lett. 259:353-356.
  • Yang C.-C. et al., 2003, Clin. Genet. 63:205-209.
  • Verovnik F. et al.,2004, Eur. J. Hum. Genet. 12:678-681.
  • Nance C.S. et al., 2006, Arch. Neurol. 63:453-457.
  • Comparison of intracellular and secretion-based strategies for production of human ¿-galactosidase A in the filamentous fungus Trichoderma reesei
    Author
    Smith, W;Jäntti, J;Oja, M;Saloheimo, M;
    Year
    2014
    Journal
    BMC Biotechnol.
    Application
    standard
  • Substrate and Substrate-Mimetic Chaperone Binding Sites in Human α-Galactosidase A Revealed by Affinity-Mass Spectrometry
    Author
    Moise, A;Maeser, S;Rawer, S;Eggers, F;Murphy, M;Bornheim, J;Przybylski, M;
    Year
    2016
    Journal
    J. Am. Soc. Mass Spectrom.
    Application
    enzyme
  • Antibody Epitope of human α-Galactosidase A revealed by affinity-mass spectrometry: A basis for reversing immunoreactivity in enzyme replacement therapy of Fabry's Disease
    Author
    Przybylski, M;
    Year
    2018
    Journal
    ChemMedChem
    Application
    SPR
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