验证数据展示
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.
Tested Applications | WB, IHC, IF-P, IP, Indirect ELISA Application Description |
Tested Reactivity | human, mouse, rat |
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 Only |
Storage Conditions | 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).