Disordered breathing in a mouse model of Dravet syndrome.
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ABSTRACT:
SUBMITTER: Kuo FS
PROVIDER: S-EPMC6506208 | biostudies-literature | 2019 Apr
REPOSITORIES: biostudies-literature
Publications
Disordered breathing in a mouse model of Dravet syndrome.
Kuo Fu-Shan FS Cleary Colin M CM LoTurco Joseph J JJ Chen Xinnian X Mulkey Daniel K DK
eLife 20190426
Dravet syndrome (DS) is a form of epilepsy with a high incidence of sudden unexpected death in epilepsy (SUDEP). Respiratory failure is a leading cause of SUDEP, and DS patients' frequently exhibit disordered breathing. Despite this, mechanisms underlying respiratory dysfunction in DS are unknown. We found that mice expressing a DS-associated <i>Scn1a</i> missense mutation (A1783V) conditionally in inhibitory neurons (<i>Slc32a1</i><sup>cre/+</sup><i>::Scn1a<sup>A1783V fl/+</sup></i>; defined as ...[more]
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