GALE rabbit pAb
Ucallm_WP16582-50
Price range: $268.00 through $328.00
Product details
Background:
This gene encodes UDP-galactose-4-epimerase which catalyzes two distinct but analogous reactions: the epimerization of UDP-glucose to UDP-galactose, and the epimerization of UDP-N-acetylglucosamine to UDP-N-acetylgalactosamine. The bifunctional nature of the enzyme has the important metabolic consequence that mutant cells (or individuals) are dependent not only on exogenous galactose, but also on exogenous N-acetylgalactosamine as a necessary precursor for the synthesis of glycoproteins and glycolipids. Mutations in this gene result in epimerase-deficiency galactosemia, also referred to as galactosemia type 3, a disease characterized by liver damage, early-onset cataracts, deafness and mental retardation, with symptoms ranging from mild ('peripheral' form) to severe ('generalized' form). Multiple alternatively spliced transcripts encoding the same protein have been identified. [provided by RefSeq, Jul 2008],
Specifications
| Size | 50μL/100μL |
| Target | GALE |
| Reactivity | Human, Mouse, Rat |
| Applications | WB |
| MW(Calculated) | 38kD |
| MW(Observed) | |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-2000 |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| Immunogen | Synthesized peptide derived from human GALE AA range: 104-154 |
| Specificity | This antibody detects endogenous levels of GALE at Human/Mouse/Rat |
| Gene Name | GALE |
| Protein Name | GALE |
| Other Name | |
| Species | Human |
| Gene ID | 2582 |
| Uniprot | Q14376, |
| Species.1 | Mouse |
| Gene ID.1 | 74246 |
| Uniprot.1 | Q8R059, |
| Species.2 | Rat |
| Gene ID.2 | |
| Uniprot.2 | P18645 |
| Cellular Localization | cytosol,extracellular exosome, |
| Tissue Expression | |
| Signaling_pathway | |
| Research Areas | >>Galactose metabolism; / >>Amino sugar and nucleotide sugar metabolism; / >>Metabolic pathways; / >>Biosynthesis of nucleotide sugars |
| Function | Catalytic activity:UDP-glucose = UDP-galactose.,cofactor:NAD.,Disease:Defects in GALE are the cause of epimerase-deficiency galactosemia (EDG) [MIM:230350]; also known as galactosemia type 3. Clinical features include early-onset cataracts, liver damage, deafness and mental retardation. There are two clinically distinct forms of EDG. (1) A benign, or 'peripheral' form with no detectable GALE activity in red blood cells and characterized by mild symptoms. Some patients may suffer no symptoms beyond raised levels of galactose-1-phosphate in the blood. (2) A much rarer 'generalized' form with undetectable levels of GALE activity in all tissues and resulting in severe features such as restricted growth and mental development.,Function:Catalyzes two distinct but analogous reactions: the epimerization of UDP-glucose to UDP-galactose and the epimerization of UDP-N-acetylglucosamine to UDP-N-acetylgalactosamine.,pathway:Carbohydrate metabolism; galactose metabolism.,similarity:Belongs to the sugar epimerase family.,subunit:Homodimer., |
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