Rabbit Anti-Galactocerebrosidase antibody

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Gacy; Galc; Galactocerebroside beta galactosidase; Galactosylceramide beta-galactosidase; Galc; GALCERase; Twitcher; GALC_HUMAN; Galactocerebroside.

Cat:

SL4691R

Species Reactivity：

Rat,(predicted: Human,Mouse,Dog,Pig,Cow,Horse,Rabbit,Guinea Pig,)

Immunogen：

KLH conjugated synthetic peptide derived from human Galactocerebroside:301-400/685

Format：

Liquid

Storage instructions：

Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

WB=1:500-2000ELISA=1:5000-10000not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Product Overview：

Sample: Brain (Rat) Lysate at 40 ugLiver (Rat) Lysate at 40 ugPrimary: Anti-Galactocerebroside (SL4691R) at 1/300 dilutionSecondary: HRP conjugated Goat-Anti-rabbit IgG (SL0295G-HRP) at 1/5000 dilutionPredicted band size: 71 kDObserved band size: 71 kD

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Unit:

Price: $228.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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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.

Function:
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.

Subcellular Location:
Lysosomal.

Tissue Specificity:
Highest level of activity in testes compared to brain, kidney, placenta and liver. Can also be found in urine.

Similarity:
Belongs to the glycosyl hydrolase 59 family.

SWISS:
P5963

Gene ID:
2581

Picture

Sample:
Brain (Rat) Lysate at 40 ug
Liver (Rat) Lysate at 40 ug
Primary: Anti-Galactocerebroside (SL4691R) at 1/300 dilution
Secondary: HRP conjugated Goat-Anti-rabbit IgG (SL0295G-HRP) at 1/5000 dilution
Predicted band size: 71 kD
Observed band size: 71 kD

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