Ultrasensitive in situ visualization of active glucocerebrosidase molecules.

@article{Witte2010UltrasensitiveIS,
  title={Ultrasensitive in situ visualization of active glucocerebrosidase molecules.},
  author={Martin D Witte and Wouter W Kallemeijn and Jan Aten and Kah-Yee Li and Anneke Strijland and Wilma E. Donker-Koopman and Adrianus M. C. H. van den Nieuwendijk and Boris Bleijlevens and Gertjan Kramer and Bogdan I Florea and Berend Hooibrink and Carla E. M. Hollak and Roelof Ottenhoff and Rolf G. Boot and Gijsbert A. van der Marel and Herman S Overkleeft and Johannes M F G Aerts},
  journal={Nature chemical biology},
  year={2010},
  volume={6 12},
  pages={
          907-13
        }
}
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… CONTINUE READING

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