MarkerGene™ Lysosomal Galactocerebrosidase (GALC) Analysis Kit

Product ID: M2774



Unit SizePriceQuantity 
1 kit
$298.88
  • Buy 5 for $239.11 each and save 20%

Availability: In stock


Description

This kit offers an easy to use protocol for detecting levels of lysosomal galactocerebrosidase in many cells types (adherent or non-adherent).

Krabbe Disease is an autosomal recessive disorder that results from a deficiency in an enzyme known as galactocerebrosidase (galactosylceramidase, GALC). It is also called Globoid Cell Leukodystrophy. This name derives from the characteristic pathology of Krabbe Disease, where macrophages accumulate high levels of undegraded galactolipids as a result of the lack of GALC activity. These cells produce a characteristic morphology difference from healthy cells, and are often termed globoid cells. The MarkerGene™ Lysosomal Galactocerebrosidase Analysis kit offers an easy to use protocol for detecting levels of lysosomal galactocerebrosidase in many cells types (adherent or non-adherent) using a lysate method and a specific lipidic fluorogenic substrate.

Technical Data
SKU M2774
Unit Size 1 kit
Alternative Names GALC Analysis Kit
Detection Method Fluorescence
Storage Conditions See individual vials for storage conditions

Other Products You Might Like
MarkerGene™ LysoLive™ Lysosomal β-Galactosidase Assay Kit

MarkerGene™ LysoLive™ Lysosomal β-Galactosidase Assay Kit

$408.43
     

References and Citations

References:

  • Wiederschain G, Srinivasa R, Kolodny E. (1992) "Characterization of 6-hexadecanoylamino-4-methylumbelliferyl-β-d-galactopyranoside as fluorogenic substrate of galactocerebrosidase for the diagnosis of Krabbe disease" Clin Chim Acta 205(1-2):87–96.
  • Svennerholm L, Vanier MT, Håkansson G. (1981) "Use of leukocytes in diagnosis of Krabbe disease and detection of carriers." Clinica Chimica 112 (1981) 333-342.
  • Kolodny E, Mumford R. (1976) "Human leukocyte acid hydrolases: Characterization of eleven lysosomal enzymes and study of reaction conditions for their automated analysis" Clin Chim Acta 70(2):247–257.
  • Suzuki K, Suzuki Y. (1970) "Globoid cell leucodystrophy (Krabbe’s disease): Deficiency of galactocerebroside -galactosidase." Proceedings of the National Academy of Sciences 66(2):302–309.
>Show more
>Show less

Click here for Newsletter articles about this product



Technical Support

Question about this product? Ask a Scientist!

We pride ourselves on the high quality of our products and want you to get the best possible results from your assays. If you have any questions about this product or need help optimizing your protocol check out the product FAQs below or ask your own question and one of our expert scientists will get back to you asap:


There are currently no frequently asked questions for this product, click the button above to ask a question