Galactosylceramidase Polyclonal Antibody, AbBy Fluor-750 Conjugated
Applications
Reactivity
Predicted Reactivity
| Overview | |
| Catalog # | bs-20477R-BF750 |
| Product Name | Galactosylceramidase Polyclonal Antibody, AbBy Fluor-750 Conjugated |
| Applications |
IF(ICC)
IF
|
| Reactivity | Human, Rat |
| Predicted Reactivity | Mouse, Dog, Cow, Sheep, Pig, Horse, Rabbit |
| Specifications | |
| Conjugation | AbBy Fluor-750 |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human Galactosylceramidase |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1ug/ul |
| Purification | Purified by Protein A. |
| Storage Buffer | Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Storage Condition | Store at -20C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. |
| Target | |
| Gene ID | 2581 |
| Swiss Prot | P54803 |
| Synonyms | Gacy; Galactocerebrosidase; Galactocerebroside beta galactosidase; Galactosylceramide beta galactosidase; galactosylceraminidase; Galc; GALCERase; Twitcher; GALC_HUMAN. |
| Background | GALC is a lysosomal enzyme that hydrolyzes galactose ester bonds in various galactolipids, including galactosylceramide, galactosylsphingosine, lactosylceramide and monogalactosyldiglyceride. Galactolipids contain glucose and/or galactose, and are found in the brain and other nerve tissue, especially the myelin sheath. Galactosylceramide is a major lipid in myelin, kidney, and epithelial cells of the small intestine and colon. Mutations in the GALC gene that compromise protein function correlate to Krabbe disease (globoid cell leukodystrophy, GLD). GLD is an autosomal recessive condition that affects approximately 1 in 150,000 infants and results in progressive destruction of the nervous system. The twitcher mouse is a model system for GLD; the genotype is a premature stop codon (W339X) in the galactosylceramidase (GALC) gene that abolishes enzymatic activity. Alternate transcriptional splice variants, encoding different isoforms, have been characterized. |
| Application Dilution | |
| IF(ICC) | |
| IF | |
Frequently asked questions
Which applications is Galactosylceramidase Polyclonal Antibody, AbBy Fluor-750 Conjugated (bs-20477R-BF750) validated for, and at what dilution?
bs-20477R-BF750 is validated for IF(ICC), IF.
Which species does Galactosylceramidase Polyclonal Antibody, AbBy Fluor-750 Conjugated (bs-20477R-BF750) react with?
Tested reactivity: Human, Rat. Predicted reactivity (expected from sequence homology but not yet tested): Mouse, Dog, Cow, Sheep, Pig, Horse, Rabbit.
How should bs-20477R-BF750 be stored?
Store at -20C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. Storage buffer: Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
What immunogen was used to raise Galactosylceramidase Polyclonal Antibody, AbBy Fluor-750 Conjugated (bs-20477R-BF750)?
KLH conjugated synthetic peptide derived from human Galactosylceramidase