catalog number :
MBS604025
products full name :
Mouse anti-Bovine Galactocerebroside
products short name :
[Galactocerebroside]
products name syn :
[Anti -Galactocerebroside]
other names :
[galactocerebrosidase isoform d; Galactocerebrosidase; galactocerebrosidase; GALCERase; galactosylceraminidase; galactocerebroside beta-galactosidase; galactosylceramide beta-galactosidase; galactosylceramidase; Galactocerebroside beta-galactosidase; Galactosylceramidase; Galactosylceramide beta-galactosidase]
other gene names :
[GALC; GALC; GALCERase]
uniprot entry name :
GALC_HUMAN
reactivity :
Bovine, Human, Mouse, Rabbit, Rat
specificity :
Recognizes bovine Galactocerebroside (GalC), sulfatide, psychosine and other galactolipids. Binds specifically with oligodendrocytes and Schwann cells. Crossreacts with the sulfatide ester of GalC, but to a 16-fold lesser extent. No crossreactivity with sphingosine, ceramide, mixed ganglioside or glucocerebroside. Species Crossreactivity: human, rat, rabbit and mouse.
purity :
Affinity Purified. Purified by Protein A affinity chromatography.
form :
Supplied as a liquid in PBS, pH 7.6, 0.09% sodium azide.
storage stability :
May be stored at 4 degree C. For long term storage, aliquot and store at 4 degree C. Do not freeze. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial prior to removing the cap. Further dilutions can be made in assay buffer.
tested application :
ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC)
app notes :
Suitable for use in Western Blot, ELISA, Immunohistochemistry and Immunocytochemistry. Dilution: Western Blot: 1:500 on 10ug PC lysates. ELISA: Used with purified galactocerebrosides. Immunohistochemistry: 0.5-10ug/ml on formalin-fixed frozen sections. Cannot be used on paraffin-embedded tissue sections since the antigen is denatured during embedding and paraffin removal. Immunocytochemistry: 0.5-10ug/ml on 4% paraformaldehyde, acetic acid or ethanol-fixed cultured cells.
image1 heading :
Western Blot (WB)
other info1 :
Immunogen: Synaptic plasma membranes from bovine hippocampus (Accession# NM_000153.2).
other info2 :
Positive Control: Neonatal cortex
products categories :
Antibodies; Abs to Proteins
products description :
Galactocerebroside (GalC) is a major galactosphingolipid of myelin which plays a role in myelination. GalC is a very useful, specific marker for oligodendroglial lineage.
ncbi acc num :
NM_000153.2
ncbi gb acc num :
NM_001201402.1
ncbi pathways :
Glycosphingolipid Metabolism Pathway (530751); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolism Pathway (477135); Metabolism Of Lipids And Lipoproteins Pathway (160976); Sphingolipid Metabolism Pathway (82994); Sphingolipid Metabolism Pathway (119543); Sphingolipid Metabolism Pathway (369)
ncbi summary :
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]
uniprot summary :
GALC: Hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon. Defects in GALC are the cause of leukodystrophy globoid cell (GLD); 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 cerebral hyperpyrexia before 2 years of age. However, a significant number of cases with later onset, presenting with unexplained blindness, weakness and/or progressive motor, and sensory neuropathy that can progress to severe mental incapacity and death, have been identified. Belongs to the glycosyl hydrolase 59 family. 4 isoforms of the human protein are produced by alternative splicing. Protein type: Lipid Metabolism - sphingolipid; Hydrolase; EC 3.2.1.46. Chromosomal Location of Human Ortholog: 14q31. Cellular Component: lysosomal lumen; lysosome. Molecular Function: galactosylceramidase activity. Biological Process: sphingolipid metabolic process; carbohydrate metabolic process; glycosphingolipid metabolic process; galactosylceramide catabolic process. Disease: Krabbe Disease