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Antibody Product Overview
| ELK.No | ES16282 |
| Product name | GALC rabbit pAb |
| Reactivity | Human; Mouse |
| Applications | WB |
| Other name | |
| Size | 50μL |
| Unit price ($) | 148 |
| Human gene ID | 2581 |
| Human Swiss-Prot | P54803 |
| Source | Rabbit |
| Isotype | IgG |
| Target | GALC |
| Fields | >>Sphingolipid metabolism;>>Metabolic pathways;>>Lysosome |
| Gene name | GALC |
| Protein name | GALC |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 14420 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | P54818 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | |
| Rat gene link | |
| Rat Swiss-Prot | |
| Rat Swiss link | |
| Immunogen | Synthesized peptide derived from human GALC AA range: 481-531 |
| Specificity | This antibody detects endogenous levels of GALC at Human/Mouse |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1：500-2000 |
| Purification | The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | 75kD |
| Observed band (KD) | |
| Background | This gene encodes a lysosomal protein which hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Mutations in this gene have been associated with Krabbe disease, also known as globoid cell leukodystrophy. Alternate transcriptional splice variants, encoding different isoforms, have been characterized. [provided by RefSeq, Jul 2008], |
| Function | catalytic activity:D-galactosyl-N-acylsphingosine + H(2)O = D-galactose + N-acylsphingosine.,caution:It is uncertain whether Met-1 or Met-17 is the initiator.,disease:Defects in GALC are the cause of leukodystrophy globoid cell (GLD) [MIM:245200]; also known as Krabbe disease. This autosomal recessive disorder results in the insufficient catabolism of several galactolipids that are important in the production of normal myelin. Clinically, the most frequent form is the infantile form. Most patients (90%) present before six months of age with irritability, spasticity, arrest of motor and mental development, and bouts of temperature elevation without infection. This is followed by myoclonic jerks of arms and legs, oposthotonus, hypertonic fits, and mental regression, which progresses to a severe decerebrate condition with no voluntary movements and death from respiratory infections or cereb |
| Subcellular location | Lysosome. |
| Expression | Detected in urine. Detected in testis, brain and placenta (at protein level). Detected in kidney and liver. |
Additional Images
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Western blot analysis of lysates from HpeG2 cells, primary antibody was diluted at 1:1000, 4°over night
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: AO-06-ES16282-50
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