验证数据展示
经过测试的应用
Positive IF-P detected in | mouse brain tissue |
推荐稀释比
应用 | 推荐稀释比 |
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Immunofluorescence (IF)-P | IF-P : 1:50-1:500 |
It is recommended that this reagent should be titrated in each testing system to obtain optimal results. | |
Sample-dependent, Check data in validation data gallery. |
产品信息
CL488-11991 targets Galc in IF-P applications and shows reactivity with human, mouse, rat samples.
经测试应用 | IF-P 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 | |
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 |
RRID | AB_3672523 |
偶联类型 | CoraLite® Plus 488 Fluorescent Dye |
最大激发/发射波长 | 493 nm / 522 nm |
形式 | Liquid |
纯化方式 | Antigen affinity purification |
UNIPROT ID | P54818 |
储存缓冲液 | PBS with 50% glycerol, 0.05% Proclin300, 0.5% BSA , pH 7.3 |
储存条件 | Store at -20°C. Avoid exposure to light. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage. |
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).
实验方案
Product Specific Protocols | |
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IF protocol for CL Plus 488 Galc antibody CL488-11991 | Download protocol |
Standard Protocols | |
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Click here to view our Standard Protocols |