产品概述
Galactosidase alpha (4R19) Rabbit Monoclonal Antibody
Rabbit Monoclonal Antibody
Rabbit
WB,IHC-P,ICC/IF,FC,IP,IF-P
Human
产品性能
Unconjugated
Unmodified
IgG
Monoclonal
Liquid
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Supplied in 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40%Glycerol, 0.01% New type preservative N and 0.05% BSA.
Affinity purification
免疫原
GLA
Alpha gal A; GALA; Galactosidase, alpha; GLA; Melibiase;
2717
P06280
产品应用
WB 1:1000-1:5000, IHC-P/IF-P 1:50-1:100, ICC/IF 1:50-1:500, FCM 1:50-1:100, IP 1:10-1:100
49kDa
研究背景
Defects in GLA are the cause of Fabry disease (FD) [MIM:301500]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. Catalyzes the hydrolysis of glycosphingolipids and participates in their degradation in the lysosome.
研究领域
武汉恩玑生命科技有限公司
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