Rac1 Activity Is Modulated by Huntingtin and Dysregulated in Models of Huntington's Disease.

ABSTRACT:

Background

Previous studies suggest that Huntingtin, the protein mutated in Huntington's disease (HD), is required for actin based changes in cell morphology, and undergoes stimulus induced targeting to plasma membranes where it interacts with phospholipids involved in cell signaling. The small GTPase Rac1 is a downstream target of growth factor stimulation and PI 3-kinase activity and is critical for actin dependent membrane remodeling.

Objective

To determine if Rac1 activity is impaired in HD or regulated by normal Huntingtin.

Methods

Analyses were performed in differentiated control and HD human stem cells and HD Q140/Q140 knock-in mice. Biochemical methods included SDS-PAGE, western blot, immunoprecipitation, affinity chromatography, and ELISA based Rac activity a

SUBMITTER: Tousley A 

PROVIDER: S-EPMC6398565 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature