Ageing contributes to phenotype transition in a mouse model of periodic paralysis.

ABSTRACT:

Background

Periodic paralysis (PP) is a rare genetic disorder in which ion channel mutation causes episodic paralysis in association with hyper- or hypokalaemia. An unexplained but consistent feature of PP is that a phenotype transition occurs around the age of 40, in which the severity of potassium-induced muscle weakness declines but onset of fixed, progressive weakness is reported. This phenotype transition coincides with the age at which muscle mass and optimal motor function start to decline in healthy individuals. We sought to determine if the phenotype transition in PP is linked to the normal ageing phenotype transition and to explore the mechanisms involved.

Methods

A mouse model of hyperkalaemic PP was compared with wild-type littermates across a range of ages (13-1

SUBMITTER: Suetterlin KJ 

PROVIDER: S-EPMC8837191 | biostudies-literature | 2021 Jul-Dec

REPOSITORIES: biostudies-literature