Analytical Data
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Gene name
Alpha-Galactosidase A
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简介
Alpha-Galactosidase A Protein, Human (HEK293, His), a recombinant human alpha-Galactosidase A produced in HEK293 cells, has a His tag at the C-terminus. Alpha-Galactosidase A Protein, Human (HEK293, His) is the lysosomal exoglycosidase responsible for the hydrolysis of terminal α-galactosyl residues from glycoconjugates and is the defective enzyme causing Fabry disease[1].
- Application
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Alternative Names
rHualpha-Galactosidase A, His; GLA; alpha-Galactosidase A
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Species
Human
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Source
HEK293
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Tag
C-6*His
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Purity
Greater than 90% as determined by SDS-PAGE.
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Uniprot
P06280
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Expression Region
L32-L429
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AA Sequence
LDNGLARTPTMGWLHWERFMCNLDCQEEPDSCISEKLFMEMAELMVSEGWKDAGYEYLCIDDCWMAPQRDSEGRLQADPQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTFADWGVDLLKFDGCYCDSLENLADGYKHMSLALNRTGRSIVYSCEWPLYMWPFQKPNYTEIRQYCNHWRNFADIDDSWKSIKSILDWTSFNQERIVDVAGPGGWNDPDMLVIGNFGLSWNQQVTQMALWAIMAAPLFMSNDLRHISPQAKALLQDKDVIAINQDPLGKQGYQLRQGDNFEVWERPLSGLAWAVAMINRQEIGGPRSYTIAVASLGKGVACNPACFITQLLPVKRKLGFYEWTSRLRSHINPTGTVLLQLENTMQMSLKDLLHHHHHH
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Protein Length
Full Length of Mature Protein
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Molecular Weight
50-60 kDa
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Endotoxin
< 1.0 EU per μg protein as determined by the LAL method.
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Form
Freeze-dried powder
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Buffer formulation
PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
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Reconstitution
Reconstitute in ddH2O to a concentration of 0.1-0.5 mg/mL. Do not vortex.
- Customization
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Stability Test
The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37℃ for 48h, and no obvious degradation and precipitation were observed. The loss rate isless than 8% within the expiration date under appropriate storage condition.
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Storage & Shelf Life
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.
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Shipping
In general, recombinant proteins are supplied as lyophilized powder and shipped at ambient temperature. For bulk packages, the proteins are provided as frozen liquid and shipped with blue ice, unless otherwise requested by the customer.
Quality inspection process
Related Products
Protein Description
Alpha-Galactosidase A (α-Gal A) is an enzyme crucial for the degradation of glycosphingolipids, particularly those containing galactose residues, and is linked to the rare genetic disorder Fabry disease. Patients with Fabry disease experience severe symptoms due to the accumulation of these lipid substrates, leading to vascular, renal, and cardiac complications. The research on recombinant α-Gal A has surged in recent years as a therapeutic approach to replace the deficient enzyme in affected individuals. Advancements in biotechnological methods have enabled the production of recombinant α-Gal A in various expression systems, such as yeast, bacteria, and mammalian cells, enhancing its efficacy and stability. Studies are focused on optimizing these production methods and improving the enzyme's pharmacokinetics and bioavailability. Additionally, understanding the enzyme's structure and function through techniques like X-ray crystallography and mutational analysis has aided in the development of enzyme replacement therapies. As a result, recombinant α-Gal A has proven effective in clinical trials, leading to its approval as a treatment option. Ongoing research aims to refine these therapies further and explore potential gene therapies to address the underlying genetic defect, ultimately improving the quality of life for Fabry disease patients.
