A novel role of the Batten disease gene CLN3: association with BMP synthesis.

@article{Hobert2007ANR,
  title={A novel role of the Batten disease gene CLN3: association with BMP synthesis.},
  author={Judith A. Hobert and G. E. Dawson},
  journal={Biochemical and biophysical research communications},
  year={2007},
  volume={358 1},
  pages={111-6}
}
Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) results from a deficiency of CLN3, a protein recently identified within detergent-resistant membranes (DRMs). To study the function of CLN3 within these domains we isolated DRMs from control and JNCL-brain and noted that JNCL-derived DRMs are less buoyant than control. Analysis of DRM phospholipids derived from JNCL-brain revealed a reduction of bis(monoacylglycerol)phosphate. Metabolic labeling of JNCL-fibroblasts demonstrated a reduction in the… CONTINUE READING

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