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C351

Recombinant Human alpha-Galactosidase A/GLA

10ug

1200

1080

现货

国产

C351

Recombinant Human alpha-Galactosidase A/GLA

50ug

3520

3168

现货

国产

C351

Recombinant Human alpha-Galactosidase A/GLA

500ug

12320

11088

现货

国产

C351

Recombinant Human alpha-Galactosidase A/GLA

1mg

17600

15840

现货

国产

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  • Catalog# C351
    Source HEK293
    Description Recombinant Human α-Galactosidase produced by transfected human cells is a secreted protein with sequence (Leu32-Leu429) of Human GLA fused with a polyhistidine tag at the C-terminus.
    Names Alpha-Galactosidase A, Alpha-D-Galactosidase A, Alpha-D-Galactoside Galactohydrolase, Melibiase, Agalsidase, GLA
    Accession # P06280
    Formulation Supplied as a 0.2 μm filtered solution of 20mM Tris-HCl, 150mM NaCl, pH 8.0
    Shipping The product is shipped on dry ice/ice packs.
    Storage Store at < -20°C, stable for 6 months after receipt.
    Please minimize freeze-thaw cycles.
    Purity Greater than 95% as determined by SEC-HPLC and reducing SDS-PAGE.
    Endotoxin Less than 0.1 ng/μg (1 IEU/μg).
    Amino Acid Sequence
    LDNGLARTPTMGWLHWERFMCNLDCQEEPDSCISEKLFMEMAELMVSEGWKDAGYEYLCIDDCWM APQRDSEGRLQADPQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTFAD WGVDLLKFDGCYCDSLENLADGYKHMSLALNRTGRSIVYSCEWPLYMWPFQKPNYTEIRQYCNHW RNFADIDDSWKSIKSILDWTSFNQERIVDVAGPGGWNDPDMLVIGNFGLSWNQQVTQMALWAIMA APLFMSNDLRHISPQAKALLQDKDVIAINQDPLGKQGYQLRQGDNFEVWERPLSGLAWAVAMINR QEIGGPRSYTIAVASLGKGVACNPACFITQLLPVKRKLGFYEWTSRLRSHINPTGTVLLQLENTM QMSLKDLLVDHHHHHH
    Background α-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. α-Galactosidase A can hydrolyze terminal α-galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose. Defects α-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.