Flupirtine blocks apoptosis in batten patient lymphoblasts and in human postmitotic CLN3‐ and CLN2‐deficient neurons

@article{Dhar2002FlupirtineBA,
  title={Flupirtine blocks apoptosis in batten patient lymphoblasts and in human postmitotic CLN3‐ and CLN2‐deficient neurons},
  author={Sumeer Dhar and Rhonda L. Bitting and Svetlana N. Rylova and Paul J. Jansen and Ellen M. Lockhart and Dwight D. Koeberl and Andrea Amalfitano and Rose-Mary N. Boustany},
  journal={Annals of Neurology},
  year={2002},
  volume={51}
}
Multiple gene defects cause Batten disease. Accelerated apoptosis accounts for neurodegeneration in the late infantile and juvenile forms that are due to defects in the CLN3 and CLN2 genes. Extensive neuronal death is seen in CLN2‐ and CLN3‐deficient human brain as well as in CLN6‐deficient sheep brain and retina. When neurons in late infantile and juvenile brain survive, they manage to do so by upregulating the neuroprotective molecule Bcl‐2. The CLN3 gene has antiapoptotic properties at the… 
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