Understanding cerebral L-lysine metabolism: the role of L-pipecolate metabolism in Gcdh-deficient mice as a model for glutaric aciduria type I

@article{Posset2014UnderstandingCL,
  title={Understanding cerebral L-lysine metabolism: the role of L-pipecolate metabolism in Gcdh-deficient mice as a model for glutaric aciduria type I},
  author={Roland Posset and S. Opp and E. Struys and A. V{\"o}lkl and H. Mohr and G. Hoffmann and S. K{\"o}lker and S. Sauer and J. Okun},
  journal={Journal of Inherited Metabolic Disease},
  year={2014},
  volume={38},
  pages={265-272}
}
Inherited deficiencies of the L-lysine catabolic pathway cause glutaric aciduria type I and pyridoxine-dependent epilepsy. Dietary modulation of cerebral L-lysine metabolism is thought to be an important therapeutic intervention for these diseases. To better understand cerebral L-lysine degradation, we studied in mice the two known catabolic routes — pipecolate and saccharopine pathways — using labeled stable L-lysine and brain peroxisomes purified according to a newly established protocol… Expand
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