bs-20477R-FITC

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: Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

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.

Conjugation: FITC

Excitation/ Emission: 494nm/518nm

Size: 100ul

Concentration: 1ug/ul

Applications: IF(ICC)(1:50-200)

Predicted Molecular Weight: 73 kDa

Cross Reactive Species: Human
Mouse
Rat

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