bs-20477R

DATASHEET

Host: Rabbit

Target Protein: Galactosylceramidase

Immunogen Range: 211-320/685

Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 2581

Swiss Prot: P54803

Source: KLH conjugated synthetic peptide derived from human Galactosylceramidase

Purification: Purified by Protein A.

Storage Buffer: 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Store at -20°C for 12 months.

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.

Size: 100µL

Concentration: 1ug/ul

Applications: IHC-P(1:200-400)
IHC-F(1:100-500)
IF(ICC)(1:50-200)
IF(1:50-200)

Predicted Molecular Weight: 73 kDa

Cross Reactive Species: Human
Rat

Predicted Cross Reactive Species: Mouse
Dog
Cow
Sheep
Pig
Horse
Rabbit

For research use only. Not intended for diagnostic or therapeutic use.