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Rabbit  Anti-Galactosylceramidase/AF594
Cat. Number:
bs-13251R-AF594
Quantity size:
100ul
Concentration:
1mg/ml  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.
Also known as:
Gacy; Galactocerebrosidase; Galactocerebroside beta galactosidase; Galactosylceramide beta galactosidase; galactosylceraminidase; Galc; GALCERase; Twitcher; GALC_HUMAN.
Specificity:
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Rabbit Polyclonal IgG, affinity purified by Protein A.
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Reacts with: (predicted: )
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Immunogen: KLH conjugated synthetic peptide derived from human G protein-regulated inducer of neurite outgrowth 2.
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Predicted Molecular Weight: 73kDa.
Storage:
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Store at -20 癈 for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20癈. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 癈.
Application:

Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.
Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.