- SPECIES: Human
- SOURCE SPECIES: Human Cells
- RECOMBINANT PROTEIN SEQUENCE: Leu32-Leu429
- FUSION TAG: C-6 His tag
- APPLICATION NOTE: This recombinant protein can be used for biological assays. For research use only.
- PREDICTED MOLECULAR WEIGHT: 46.39 kD
- PURITY: Greater than 95% as determined by reducing SDS-PAGE.
Endotoxin level less than 0.1 ng/ug (1 IEU/ug) as determined by LAL test.
- PHYSICAL STATE: Liquid
- BUFFER: Supplied as a 0.2 um filtered solution of 20mM TrisHCl, 150mM NaCl, pH 8.0. It is not recommended to reconstitute to a concentration less than 100 ug/ml.
- STORAGE CONDITIONS: Store at -20°C, stable for 6 months after receipt.
Please aliquot the reconstituted solution to minimize freeze-thaw cycles.
- NCBI OFFICIAL SYMBOL: GLA
- ADDITIONAL NAMES: Alpha-Galactosidase A, Alpha-D-Galactosidase A, Alpha-D-Galactoside Galactohydrolase, Melibiase, Agalsidase, GLA
- Protein Accession Number: P06280
- NCBI GENE ID NUMBER: 2717
- alpha-Galactosidase A is a homodimeric glycoprotein that belongs to the glycosyl hydrolase 27 family. It is a lysosomal enzyme and used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. alpha-Galactosidase A can hydrolyze terminal alpha-galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose. Defects alpha-Galactosidase A are the cause of Fabry disease (FD) which is a rare X-linked sphingolipidosis disease with 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. 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.
- FOR RESEARCH USE ONLY
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- Disclaimer: Products are intended for laboratory research purposes only and should be used by qualified personnel only. They are not intended for use in humans. ProSci is not liable for damages or injuries resulting from receipt and/or use of ProSci materials. Please refer to the Material Safety Data Sheet (MSDS) for safe storage, handling, and use procedures.
CATALOG NUMBER: 91-323
- Size: 0.05 mg
- List Price: $525.00
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