These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

  • Title: Ultrasensitive in situ visualization of active glucocerebrosidase molecules.
    Author: Witte MD, Kallemeijn WW, Aten J, Li KY, Strijland A, Donker-Koopman WE, van den Nieuwendijk AM, Bleijlevens B, Kramer G, Florea BI, Hooibrink B, Hollak CE, Ottenhoff R, Boot RG, van der Marel GA, Overkleeft HS, Aerts JM.
    Journal: Nat Chem Biol; 2010 Dec; 6(12):907-13. PubMed ID: 21079602.
    Abstract:
    Deficiency of glucocerebrosidase (GBA) underlies Gaucher disease, a common lysosomal storage disorder. Carriership for Gaucher disease has recently been identified as major risk for parkinsonism. Presently, no method exists to visualize active GBA molecules in situ. We here report the design, synthesis and application of two fluorescent activity-based probes allowing highly specific labeling of active GBA molecules in vitro and in cultured cells and mice in vivo. Detection of in vitro labeled recombinant GBA on slab gels after electrophoresis is in the low attomolar range. Using cell or tissue lysates, we obtained exclusive labeling of GBA molecules. We present evidence from fluorescence-activated cell sorting analysis, fluorescence microscopy and pulse-chase experiments of highly efficient labeling of GBA molecules in intact cells as well as tissues of mice. In addition, we illustrate the use of the fluorescent probes to study inhibitors and tentative chaperones in living cells.
    [Abstract] [Full Text] [Related] [New Search]