验证数据展示
经过测试的应用
| Positive WB detected in | A375 cells, A549 cells, SH-SY5Y cells, mouse brain tissue, rat brain tissue |
| Positive IP detected in | NIH/3T3 cells |
| Positive IHC detected in | human gliomas tissue Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0 |
| Positive IF-P detected in | mouse brain tissue |
推荐稀释比
| Application | Dilution |
|---|---|
| Western Blot (WB) | WB : 1:500-1:1000 |
| Immunoprecipitation (IP) | IP : 0.5-4.0 ug for 1.0-3.0 mg of total protein lysate |
| Immunohistochemistry (IHC) | IHC : 1:20-1:200 |
| 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
11991-1-AP targets Galc in WB, IHC, IF-P, IP, ELISA applications and shows reactivity with human, mouse, rat samples.
| Tested Applications | WB, IHC, IF-P, IP, ELISA Application Description |
| Cited Applications | WB, IHC, IF |
| Tested Reactivity | human, mouse, rat |
| Cited Reactivity | human, mouse, rat, zebrafish |
| Immunogen | Galc fusion protein Ag3914 种属同源性预测 |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Full Name | galactosylceramidase |
| Synonyms | GALCERase, Galactosylceramide beta-galactosidase, Galactocerebroside beta-galactosidase, Galactocerebrosidase, Gacy |
| Calculated Molecular Weight | 77 kDa |
| Observed Molecular Weight | 80 kDa, 30 kDa, 50 kDa |
| GenBank Accession Number | BC086671 |
| Gene Symbol | Galc |
| Gene ID (NCBI) | 14420 |
| RRID | AB_10641987 |
| Conjugate | Unconjugated |
| Form | Liquid |
| Purification Method | Antigen affinity purification |
| UNIPROT ID | P54818 |
| Storage Buffer | PBS with 0.02% sodium azide and 50% glycerol pH 7.3. |
| Storage Conditions | Store at -20°C. 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 | |
|---|---|
| WB protocol for Galc antibody 11991-1-AP | Download protocol |
| IHC protocol for Galc antibody 11991-1-AP | Download protocol |
| IF protocol for Galc antibody 11991-1-AP | Download protocol |
| IP protocol for Galc antibody 11991-1-AP | Download protocol |
| Standard Protocols | |
|---|---|
| Click here to view our Standard Protocols |
发表文章
| Species | Application | Title |
|---|---|---|
Dev Cell NPC1-mTORC1 Signaling Couples Cholesterol Sensing to Organelle Homeostasis and Is a Targetable Pathway in Niemann-Pick Type C. | ||
Stem Cells Multipotent Stromal Cells Alleviate Inflammation, Neuropathology, and Symptoms Associated with Globoid Cell Leukodystrophy in the Twitcher Mouse. | ||
Mol Ther AAV-mediated expression of galactocerebrosidase in brain results in attenuated symptoms and extended life span in murine models of globoid cell leukodystrophy. | ||
Mol Ther Extended normal life after AAVrh10-mediated gene therapy in the mouse model of Krabbe disease. | ||
Glia Ncx3 gene ablation impairs oligodendrocyte precursor response and increases susceptibility to experimental autoimmune encephalomyelitis. | ||
FASEB J Polyunsaturated fatty acid deficiency affects sulfatides and other sulfated glycans in lysosomes through autophagy-mediated degradation. |