GLa (Galactosidase Alpha, GALA, GL-A, Alpha-D-Galactoside Galactohydrolase, Alpha-D-Galactosidase A, Melibiase, Agalsidase)

Size

£581.00

SKU:: 363300
Purity:: Purified by immunoaffinity chromatography.
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Rabbit
Immunogen:: Recombinant protein corresponding to Val29-Gly258 of mouse GLa, expressed in E. coli.
Format:: Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
Extra Details:: Alpha-galactosidase is a glycoside hydrolase enzyme that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. It is encoded by the GLA gene. This enzyme is a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. It predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry's disease, a rare lysosomal storage disorder and sphingolipidosis that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties. Genzyme produces synthetic agalsidase beta under the brand name Fabrazyme for treatment of Fabry's disease. Applications: Suitable for use in ELISA, Western Blot, Immunohistochemistry, Immunocytochemistry. Other applications not tested. Recommended Dilution: ELISA: 1:100-1:5000 Western Blot: 1:50-400 Immunohistochemistry (frozen): 1:50-500 Immunohistochemistry (paraffin): 1:10-100 Immunocytochemistry: 1:50-500 Optimal dilutions to be determined by the researcher. AA Sequence: MGSSHHHHHH SSGLVPRGSH MASMTGGQQM GRGSEF- VR ALDNGLARTP TMGWLHWERF MCNLDCQEEP DACISEQLFM QMAELMVSDG WRDAGYDYLC IDDCWMAPER DSKGRLQADP QRFPSGIKHL ANYVHSKGLK LGIYADVGNK TCAGFPGSFG SYDIDAQTFA DWGVDLLKFD GCHCDSVVSL ENGYKYMALA LNRTGRSIVY SCEWPLYLRP FHKPNYTDIQ YYCNHWRNFD DVYDSWESIK NILSWTVVYQ KEIVEVAG Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Shipping Conditions:: Blue Ice