Transient neuromotor phenotype in transgenic spastic mice expressing low levels of glycine receptor β-subunit: an animal model of startle disease

@inproceedings{Becker2000TransientNP,
  title={Transient neuromotor phenotype in transgenic spastic mice expressing low levels of glycine receptor β-subunit: an animal model of startle disease},
  author={Lore Becker and Bettina Hartenstein and Johannes Schenkel and Jochen Kuhse and Heinrich Betz and Hans Jochem Weiher},
  booktitle={The European journal of neuroscience},
  year={2000}
}
Startle disease or hereditary hyperekplexia has been shown to result from mutations in the alpha1-subunit gene of the inhibitory glycine receptor (GlyR). In hyperekplexia patients, neuromotor symptoms generally become apparent at birth, improve with age, and often disappear in adulthood. Loss-of-function mutations of GlyR alpha or beta-subunits in mice show rather severe neuromotor phenotypes. Here, we generated mutant mice with a transient neuromotor deficiency by introducing a GlyR beta… CONTINUE READING

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