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Description
Galactosylceramidase Recombinant Rabbit mAb (S-3402-17)Product Specification Host Rabbit Antigen Galactosylceramidase Synonyms Galactocerebrosidase; GALCERase; Galactocerebroside beta galactosidase; Galactosylceramide beta galactosidase; Galc Immunogen Recombinant Protein Location Lysosome Accession P54818 Clone Number S 3402 17 Antibody Type Recombinant mAb Isotype IgG Application WB, IHC P Reactivity Hu, Ms, Rt, Mk Positive Sample A549, SH SY5Y, A375, HepG2, mouse brain, COS 7 Purification Protein A
Product Specification
| Host | Rabbit |
| Antigen | Galactosylceramidase |
| Synonyms | Galactocerebrosidase; GALCERase; Galactocerebroside beta-galactosidase; Galactosylceramide beta-galactosidase; Galc |
| Immunogen | Recombinant Protein |
| Location | Lysosome |
| Accession | P54818 |
| Clone Number | S-3402-17 |
| Antibody Type | Recombinant mAb |
| Isotype | IgG |
| Application | WB, IHC-P |
| Reactivity | Hu, Ms, Rt, Mk |
| Positive Sample | A549, SH-SY5Y, A375, HepG2, mouse brain, COS-7 |
| Purification | Protein A |
| Concentration | 0.5 mg/ml |
| Conjugation | Unconjugated |
| Physical Appearance | Liquid |
| Storage Buffer | PBS, 40% Glycerol, 0.05% BSA, 0.03% Proclin 300 |
| Stability & Storage | 12 months from date of receipt / reconstitution, -20 °C as supplied |
Dilution
| application | dilution | species |
| WB | 1:1000 | Hu, Ms, Mk |
| IHC-P | 1:1000 | Hu, Ms, Rt |
Background
Galactosylceramidase, also known as galactocerebrosidase (GALC), is a lysosomal enzyme encoded by the GALC gene on chromosome 14q31.3 that catalyzes the hydrolytic cleavage of galactose from galactosylceramide and other galactolipids, playing a crucial role in myelin maintenance and turnover; deficiency in this enzyme leads to the accumulation of toxic metabolites, particularly psychosine, resulting in Krabbe disease (globoid cell leukodystrophy), a severe neurodegenerative disorder characterized by demyelination, multinucleated globoid cells in the white matter, and progressive loss of motor and cognitive functions, with early-onset forms typically manifesting within the first six months of life and proving fatal within a few years.
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