Motor neuropathy in porphobilinogen deaminase-deficient mice imitates the peripheral neuropathy of human acute porphyria.

@article{Lindberg1999MotorNI,
  title={Motor neuropathy in porphobilinogen deaminase-deficient mice imitates the peripheral neuropathy of human acute porphyria.},
  author={Raija L. P. Lindberg and Roland Martini and Melanie Baumgartner and Beat Erne and Jan van der Borg and J{\"u}rgen Zielasek and Kenneth Ricker and A. Steck and Klaus Viktor Toyka and Urs A. Meyer},
  journal={The Journal of clinical investigation},
  year={1999},
  volume={103 8},
  pages={1127-34}
}
Acute porphyrias are inherited disorders caused by partial deficiency of specific heme biosynthesis enzymes. Clinically, porphyrias are manifested by a neuropsychiatric syndrome that includes peripheral neuropathy. Although much is known about the porphyrias' enzyme defects and their biochemical consequences, the cause of the neurological manifestations remains unresolved. We have studied porphyric neuropathy in mice with a partial deficiency of porphobilinogen deaminase (PBGD). PBGD-deficient… CONTINUE READING

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Porphyrical neuropathy. In Peripheric neuropathy

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