Lysosomal Galactocerebrosidase (GALC) Analysis Kit (ab253371)
Key features and details
- Detection method: Fluorescent
Overview
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Product name
Lysosomal Galactocerebrosidase (GALC) Analysis Kit -
Detection method
Fluorescent -
Product overview
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 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.
This kit is sold by Marker Gene under product code M2774.
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Notes
Abcam has not and does not intend to apply for the REACH Authorisation of customers’ uses of products that contain European Authorisation list (Annex XIV) substances.
It is the responsibility of our customers to check the necessity of application of REACH Authorisation, and any other relevant authorisations, for their intended uses.
Properties
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Storage instructions
Please refer to protocols. -
Components 1 units Lysis Buffer A 1 x 20ml Lysis Buffer B 1 x 20ml Reaction Buffer 1 x 5ml Stop Buffer 1 x 20ml Substrate Reagent (Light sensitive) 3 x 10 vials -
Research areas
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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. -
Tissue specificity
Detected in urine. Detected in testis, brain and placenta (at protein level). Detected in kidney and liver. -
Involvement in disease
Leukodystrophy, globoid cell -
Sequence similarities
Belongs to the glycosyl hydrolase 59 family. -
Cellular localization
Lysosome. - Information by UniProt
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Alternative names
- Gacy
- Galactocerebrosidase
- Galactocerebroside beta galactosidase
see all
Images
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Leukocytes derived from a healthy individual and from a Krabbe patient.
Fluorescence was recorded using a Tecan Infinite M200 Pro plate reader, blank readings were subtracted from samples and an average of 6 wells presented.
