BP133 Galactocerebroside antibody

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0.1 ml / €450.00
Please visit the country specific website of Acris Antibodies or contact your local Distributor to buy this product.

Quick Overview

Rabbit anti Bovine, Human, Mouse, Rat Galactocerebroside

BP133

Product Description for Galactocerebroside

Rabbit anti Bovine, Human, Mouse, Rat Galactocerebroside.
Presentation: Serum
Product is tested for Frozen Sections, Enzyme Immunoassay.

Properties for Galactocerebroside

Product Category Primary Antibodies
Quantity 0.1 ml
Presentation Serum
Reactivity Bov, Hu, Ms, Rt
Applications C, E
Clonality Polyclonal
Host Rabbit
Shipping to Worldwide
PDF datasheet View Datasheet
Manufacturer Acris Antibodies GmbH
Material safety datasheet MSDS for Polyclonal Antibodies (de)

Datasheet Extract

Immunogen
Immunogen:
Galactocerebroside purified from Bovine brain.
Application ELISA: 1/50-1/100.
Immunohistochemistry on Frozen Sections: 1/10-1/50; For staining of living cells it is recommended that the antisera be heat inactived prior to using to remove complement. The recommended fixative is 4% paraformaldehyde.
Immunofluorescence: 1/10-1/50; The epitope recognised by this antibody is reported to be sensitive to formaldehyde fixation and tissue processing. We recommends the use of acetone fixation for frozen sections.
Background Galactocerebroside is a surface antigen typically found on newly differentiated or developing oligodendrocytes.
Galactosylceramidase hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. It is an 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.
It shows highest level of activity in testes compared to brain, kidney, placenta and liver. It can also be found in urine.
Defects in Galactosylceramidase are the cause of globoid cell leukodystrophy (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.
Storage Store the antibody (in aliquots) at -20 °C.
Avoid repeated freezing and thawing.
Shelf life: one year from despatch.
Format
State:
Liquid Serum without additives.
Serum
Species Reactivity
Species reactivity (expected):
Human, Rat.
Species reactivity (tested):
Bovine, Mouse.
Specificity
Specificity:
This antibody recognises Galactocerebroside, a surface antigen typically found on newly differentiated or developing oligodendrocytes.
Staining of live cells prior to fixation will show a typical "patching" appearance.
Cells fixed prior to staining will have a more solid, "smooth" appearance.

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