Application
Immunoperoxidase staining of formalin fixed, paraffin-embedded human upper stomach tissue showing cytoplasmic staining of glandular cells.Application
Western blot analysis of GALC expression in non-transfected: (A) and human GALC transfected: (B) 293T whole cell lysates.Application
Western blot analysis of GALC expression in non-transfected: (A) and human GALC transfected: (B) 293T whole cell lysates.| Product Name | GALC Antibody (H-300) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 31-330 mapping near the N-terminus of GALC of human origin |
| Modification Notes | GALC (galactosylceramidase) 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. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFIHCELISA |
| recommended for detection of GALC isoforms 1 and 2 of mouse, rat and human origin by WB, IP, IF, IHC(P) and ELISA also reactive with additional species, including equine, canine, bovine and porcine: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 31-330 mapping near the N-terminus of GALC of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 31-330 mapping near the N-terminus of GALC of human origin |
| Entrez Gene | 2581 |
|---|
Application
Immunoperoxidase staining of formalin fixed, paraffin-embedded human upper stomach tissue showing cytoplasmic staining of glandular cells.
Application
Western blot analysis of GALC expression in non-transfected: (A) and human GALC transfected: (B) 293T whole cell lysates.
Application
Western blot analysis of GALC expression in non-transfected: (A) and human GALC transfected: (B) 293T whole cell lysates.| Application Notes | recommended for detection of GALC isoforms 1 and 2 of mouse, rat and human origin by WB, IP, IF, IHC(P) and ELISA also reactive with additional species, including equine, canine, bovine and porcine: |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
