84-4418-98　GLa (Galactosidase Alpha, GALA, GL-A, Alpha-D-Galactoside Galactohydrolase, Alpha-D-Galactosidase A, Melibiase, Agalsidase) 200ul　363300

特徴

• 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: 1:100-1:5000Western Blot: 1:50-400Immunohistochemistry (frozen): 1:50-500Immunohistochemistry (paraffin): 1:10-100Immunocytochemistry: 1:50-500Optimal 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.