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Invitrogen
alpha Galactosidase Polyclonal Antibody
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alpha Galactosidase Antibody (PA5-116571) in WB
Western blot analysis of alpha Galactosidase in extracts of HeLa cells. Samples were incubated with alpha Galactosidase Polyclonal antibody (Product # PA5-116571) using a dilution of 1:1,000, followed by HRP Goat Anti-Rabbit IgG (H+L) at a dilution of 1:10,000. Lysates/proteins: 25 µg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 30s.
产品信息
PA5-116571
产品规格
种属反应
Human
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
Recombinant fusion protein containing a sequence corresponding to amino acids 150-429 of human Galactosidase alpha (Galactosidase alpha (GLA)) (NP_0001601)
偶联物
形式
Liquid
浓度
0.96 mg/mL
纯化类型
Affinity chromatography
保存液
PBS, pH 7.3, with 50% glycerol
内含物
0.02% sodium azide
保存条件
-20°C, Avoid Freeze/Thaw Cycles
运输条件
Ambient (domestic); Wet ice (international)
RRID
产品详细信息
Positive Samples: HeLa
Immunogen sequence: GYYDIDAQTF ADWGVDLLKF DGCYCDSLEN LADGYKHMSL ALNRTGRSIV YSCEWPLYMW PFQKPNYTEI RQYCNHWRNF ADIDDSWKSI KSILDWTSFN QERIVDVAGP GGWNDPDMLV IGNFGLSWNQ QVTQMALWAI MAAPLFMSND LRHISPQAKA LLQDKDVIAI NQDPLGKQGY QLRQGDNFEV WERPLSGLAW AVAMINRQEI GGPRSYTIAV ASLGKGVACN PACFITQLLP VKRKLGFYEW TSRLRSHINP TGTVLLQLEN TMQMSLKDLL
靶标信息
This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme 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 disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: agalsidase alfa; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; alpha-D-galactoside galactohydrolase 1; alpha-gal A; Alpha-galactosidase A; galactosidase, alpha; Galactosylgalactosylglucosylceramidase GLA; Melibiase
基因别名: GALA; GLA
UniProt ID: (Human) P06280
Entrez Gene ID: (Human) 2717
