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Mouse Galc Polyclonal antibody, PBS Only

Galc Polyclonal Antibody for WB, IHC, IF-P, IP, Indirect ELISA
Cat No. 11991-1-PBS

产品说明书

宿主/亚型

Rabbit / IgG

种属反应性

human, mouse, rat

应用

WB, IHC, IF-P, IP, Indirect ELISA

GALCERase, Galactosylceramide beta-galactosidase, Galactocerebroside beta-galactosidase, Galactocerebrosidase, Gacy

缓冲液配方:  PBS Only
PBS and Azide
PBS Only
偶联物:  Unconjugated
规格: 

-/ -


产品信息

11991-1-PBS targets Galc in WB, IHC, IF-P, IP, Indirect ELISA applications and shows reactivity with human, mouse, rat samples.

经测试应用 WB, IHC, IF-P, IP, Indirect ELISA Application Description
经测试反应性 human, mouse, rat
免疫原 Galc fusion protein Ag3914 种属同源性预测
宿主/亚型 Rabbit / IgG
抗体类别 Polyclonal
产品类型 Antibody
全称 galactosylceramidase
别名 GALCERase, Galactosylceramide beta-galactosidase, Galactocerebroside beta-galactosidase, Galactocerebrosidase, Gacy
计算分子量 77 kDa
观测分子量 80 kDa, 30 kDa, 50 kDa
GenBank蛋白编号BC086671
基因名称 Galc
Gene ID (NCBI) 14420
RRIDAB_10641987
偶联类型 Unconjugated
形式 Liquid
纯化方式Antigen affinity purification
UNIPROT IDP54818
储存缓冲液 PBS only , pH 7.3
储存条件Store at -80°C.
The product is shipped with ice packs. Upon receipt, store it immediately at -80°C

背景介绍

The GALC antibody targets the liposomal enzyme Galactosylceramidase (GALC), which belongs to the glycosyl hydrolase 59 family. It hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. It is primarily found in the brain and kidneys where galactolipids are hydrolyzed (PMID:8634707). Deficiencies of GALC are primarily associated with the autosomal recessive Krabbe's disease. This disease is characterized by developmental delay caused by apoptosis of myelin-forming cells. GALC is responsible for hydrolyzing galactosylceramide, a cerebroside that is an important component of myelin. A deficiency in GALC causes loss of myelin to nerve cells, resulting in delayed nerve transmissions. Krabbe's disease has varying degrees of severity due to a large number of different genetic mutations in the gene. The GALC antibody can be used to detect the deletions in the GALC gene and functions of the enzyme (PMID:20886637). Normal GALC mRNA encodes the 80 kDa precursor, which is processed into 50 and 30 kDa subunits (PMID: 26865610).

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