APP Causes Hyperexcitability in Fragile X Mice.

ABSTRACT: Amyloid-beta protein precursor (APP) and metabolite levels are altered in fragile X syndrome (FXS) patients and in the mouse model of the disorder, Fmr1^KO mice. Normalization of APP levels in Fmr1^KO mice (Fmr1^KO /APP^HET mice) rescues many disease phenotypes. Thus, APP is a potential biomarker as well as therapeutic target for FXS. Hyperexcitability is a key phenotype of FXS. Herein, we determine the effects of APP levels on hyperexcitability in Fmr1^KO brain slices. Fmr1^KO /APP^HET slices exhibit complete rescue of UP states in a neocortical hyperexcitability model and reduced duration of ictal discharges in a CA3 hippo

SUBMITTER: Westmark CJ 

PROVIDER: S-EPMC5156834 | biostudies-literature | 2016

REPOSITORIES: biostudies-literature