Progressive central cardiorespiratory rate downregulation and intensifying epilepsy lead to sudden unexpected death in epilepsy in mouse model of the most common human ATP1A3 mutation.

ABSTRACT:

Objective

This study was undertaken to test the following hypotheses in the Atp1a3^Mashl/+ mouse (which carries the most common human ATP1A3 (the major subunit of the neuronal Na⁺/K⁺-adenosine triphosphatase [ATPase]) mutation, D801N): sudden unexpected death in epilepsy (SUDEP) occurs during seizures and is due to terminal apneas in some and due to lethal cardiac arrhythmias in others; and Atp1a3^Mashl/+ mice have central cardiorespiratory dysregulation and abnormal respiratory drive.

Methods

Comparison was made of littermate wild-type and Atp1a3^Mashl/+ groups using (1) simultaneous in vivo video-telemetry recordings of electroencephalogram, electrocardiogram, and breathing; (2) whole-body plethysmography; and (3) hypoglo

SUBMITTER: Hunanyan AS 

PROVIDER: S-EPMC11908895 | biostudies-literature | 2025 Mar

REPOSITORIES: biostudies-literature