产品中心
-
产品货号
产品名称
规格
销售价
会员价
货期
产地
订购
-
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
- 产品简介
- 使用说明
- 相关产品
- 参考文献
- 购买咨询
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 QMSLKDLLVDHHHHHHBackground 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.