PLS3 Overexpression Delays Ataxia in Chp1 Mutant Mice.

ABSTRACT: Many neurodegenerative disorders share common pathogenic pathways such as endocytic defects, Ca²⁺ misregulation and defects in actin dynamics. Factors acting on these shared pathways are highly interesting as a therapeutic target. Plastin 3 (PLS3), a proven protective modifier of spinal muscular atrophy across species, is a remarkable example of the former, and thereby offers high potential as a cross-disease modifier. Importantly, PLS3 has been linked to numerous proteins associated with various neurodegenerative diseases. Among them, PLS3 directly interacts with calcineurin like EF-hand protein 1 (CHP1), whose loss-of-function results in ataxia. In this study, we aimed to determine whether PLS3 is a cross-disease modifier for ataxia caused by Chp1 mutation in mice. For

SUBMITTER: Janzen E 

PROVIDER: S-EPMC6761326 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature