Article
Functional recovery of glycine receptors in spastic murine model of startle disease.
Neurobiology of disease - 1 Feb 2006
Molon Annamaria, Di Giovanni Simone, Hathout Yetrib, Natale Joanne, Hoffman Eric P
Abstract excerpt
Clinical variability is common in inherited gene defects of the central nervous system in humans and in animal models of human disorders. Here, we used the homozygous spastic (spa) mutant mice, which resemble human hereditary hyperekplexia, to determine the molecular remodeling of the spinal cord through the course of the disease, and develop a model for clinical disparity between littermates. The spa mutation is...
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