Lysosomal Galactocerebrosidase (GALC) Analysis Kit ab253371 uses a simple protocol to measure levels of lysosomal galactocerebrosidase in many cells types (adherent or non-adherent).
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- Human gene therapy 33:499-5172022Efficacy and Safety of a Krabbe Disease Gene Therapy.Applications:Unspecified applicationReactive species:Unspecified reactive speciesJuliette Hordeaux,Brianne A Jeffrey,Jinlong Jian,Gourav R Choudhury,Kristofer Michalson,Thomas W Mitchell,Elizabeth L Buza,Jessica Chichester,Cecilia Dyer,Jessica Bagel,Charles H Vite,Allison M Bradbury,James M WilsonPubMed 35333110
- PloS one 11:e01563122016Fluorogenic Substrates for Visualizing Acidic Organelle Enzyme Activities.Applications:Unspecified applicationReactive species:Unspecified reactive speciesFiona Karen Harlan et. al.PubMed 27228111
Key facts
- Detection method
- Fluorescent
Target data
Function
Hydrolyzes the galactose ester bonds of glycolipids such as galactosylceramide and galactosylsphingosine (PubMed:8281145, PubMed:8399327). 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 (PubMed:8281145, PubMed:8399327).
Alternative names
Galactocerebrosidase, GALCERase, Galactocerebroside beta-galactosidase, Galactosylceramidase, Galactosylceramide beta-galactosidase, GALC
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Lysosomal Galactocerebrosidase (GALC) Analysis Kit ab253371 uses a simple protocol to measure levels of lysosomal galactocerebrosidase in many cells types (adherent or non-adherent).
Key facts
- Detection method
- Fluorescent
Storage
- Shipped at conditions
- Blue Ice
- Appropriate short-term storage conditions
- Multi
- Appropriate long-term storage conditions
- Multi
- Storage information
- Please refer to protocols
Notes
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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Supplementary info
- This supplementary information is collated from multiple sources and compiled automatically.
- Activity summary
GALC also known as galactocerebrosidase or galactosylceramidase is an enzyme with a molecular mass of about 68 kDa. It functions mechanically by hydrolyzing galactosylceramide (galactocerebroside) into galactose and ceramide. GALC expresses widely in the brain and other nervous tissues particularly in the lysosomes of glial cells contributing to the breakdown of lipids. The efficient lysosomal degradation process performed by GALC is critical for maintaining cellular lipid homeostasis.
- Biological function summary
GALC plays an important role in the metabolism of complex lipids. It functions not as part of a larger protein complex but rather as an independent enzyme acting on specific substrates like galactosylceramide. The enzyme's activity contributes to the transformation of lipids necessary for the proper structure and function of myelin in the central nervous system. By maintaining proper lipid levels GALC supports the integrity and stability of myelinated neurons.
- Pathways
GALC is involved in the sphingolipid degradation pathway contributing to the catabolic process of sphingolipids. This pathway ensures the break down and recycling of sphingolipid components like ceramide which are essential for cell signaling and membrane integrity. Another relevant pathway is the lysosomal storage pathway where GALC's enzymatic function prevents the abnormal accumulation of galactosylceramide and related substrates. GALC activity has links to other lysosomal enzymes like arylsulfatase A which also participate in lipid metabolism within the lysosome.
- Associated diseases and disorders
GALC mutations and deficiencies lead to Krabbe disease a severe lysosomal storage disorder affecting the nervous system. This pathology results from the accumulation of psychosine a toxic metabolite causing widespread demyelination. Another related condition is globoid cell leukodystrophy associated with GALC dysfunction. Proteins like myelin-associated glycoprotein may interact indirectly with GALC through these disease processes as GALC's enzymatic activity affects myelin maintenance.
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Fluorescent Cell Imaging - Lysosomal Galactocerebrosidase (GALC) Analysis Kit (ab253371)
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.
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