Functional Consequences of the Postnatal Switch From Neonatal to Mutant Adult Glycine Receptor α1 Subunits in the Shaky Mouse Model of Startle Disease

Functional Consequences of the Postnatal Switch From Neonatal to Mutant Adult Glycine Receptor α1 Subunits in the Shaky Mouse Model of Startle Disease

Mutations in GlyR α1 or β subunit genes in humans and rodents lead to severe startle disease characterized by rigidity, massive stiffness and excessive startle responses upon unexpected tactile or acoustic stimuli. The recently characterized startle disease mouse mutant shaky carries a missense muta...

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Bibliographic Details
Main Authors: Natascha Schaefer, Fang Zheng, Johannes van Brederode, Alexandra Berger, Sophie Leacock, Hiromi Hirata, Christopher J. Paige, Robert J. Harvey, Christian Alzheimer, Carmen Villmann
Format: Article
Language:English
Published: Frontiers Media S.A. 2018-05-01
Series:Frontiers in Molecular Neuroscience
Subjects:
glycine receptor
startle disease
β8-β9 loop
mouse model
fast decay
shaky
Online Access:https://www.frontiersin.org/article/10.3389/fnmol.2018.00167/full

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