Recombinant Human alpha-Galactosidase A, His (HEK293-expressed)，一種在HEK293細(xì)胞中產(chǎn)生的重組人α-半乳糖苷酶A，在 C 末端有 His 標(biāo)簽。Human alpha-Galactosidase A是負(fù)責(zé)從糖結(jié)合物中水解末端α-半乳糖基殘基的溶酶體外糖苷酶，是導(dǎo)致法布里病的有缺陷的酶。
Synonyms
rHualpha-Galactosidase A, His; GLA; alpha-Galactosidase A ; 重組人α-半乳糖苷酶 A，His (HEK293-expressed)
Species
HumanSource
HEK 293 Accession
P06280 Gene ID
2717 Molecular Weight
50-60 kDa AA Sequence
LDNGLARTPT MGWLHWERFM CNLDCQEEPD SCISEKLFME MAELMVSEGW KDAGYEYLCI DDCWMAPQRD SEGRLQADPQ RFPHGIRQLA NYVHSKGLKL GIYADVGNKT CAGFPGSFGY YDIDAQTFAD WGVDLLKFDG CYCDSLENLA DGYKHMSLAL NRTGRSIVYS CEWPLYMWPF QKPNYTEIRQ YCNHWRNFAD IDDSWKSIKS ILDWTSFNQE RIVDVAGPGG WNDPDMLVIG NFGLSWNQQV TQMALWAIMA APLFMSNDLR HISPQAKALL QDKDVIAINQ DPLGKQGYQL RQGDNFEVWE RPLSGLAWAV AMINRQEIGG PRSYTIAVAS LGKGVACNPA CFITQLLPVK RKLGFYEWTS RLRSHINPTG TVLLQLENTM QMSLKDLLHH HHHH Biological Activity
Data is not available. Appearance
Solution Formulation
Supplied as a 0.2 μm filter solution of 20 mM Tris-HCl, 150 mM NaCl, pH 8.0. Endotoxin Level
<1 EU/μg, determined by LAL method. Reconstitution
N/A Storage & Stability
Recombinant Human alpha-Galactosidase A, His (HEK293-expressed) (rHualpha-Galactosidase A, His) is stored at -20°C. It is stable at 4°C for 1 week or -20°C for longer. It is recommended to freeze aliquots at -20°C or -80°C for extended storage. Shipping
Shipping with dry ice. Background
alpha-Galactosidase A (α-GAL, also known as α-GAL A) is responsible for the breakdown of α-galactosides in the lysosome. Defects in human alpha-Galactosidase A lead to the development of Fabry disease, a lysosomal storage disorder characterized by the buildup of α-galactosylated substrates in the tissues. alpha-Galactosidase A is an active target of clinical research: there are currently two treatment options for Fabry disease, recombinant enzyme replacement therapy and pharmacological chaperone therapy[2]. |
