N471D WASH complex subunit strumpellin knock-in mice display BPTF and KLHL11 dysregulation in brain tissue

ABSTRACT: We investigated brain tissue from N471D WASH complex subunit strumpellin knock-in mice as a genetic model for hereditary spastic paraplegia type 8. While WASHC5-related protein interaction partners and complexes showed no change in abundancies, the proteomic analysis depicted consistent upregulation of BPTF and downregulation of KLHL11 in heterozygous and homozygous knock-in mice. This finding suggests mechanistic links for hereditary spastic paraplegia type 8 through the roles of BPTF and KLHL11 in neurodegeneration and protein quality control, respectively.

INSTRUMENT(S):

ORGANISM(S): Mus Musculus (mouse)

TISSUE(S): Brain

SUBMITTER: Ilka Wittig  

LAB HEAD: Andreas Schmidt

PROVIDER: PXD025315 | Pride | 2021-07-26

REPOSITORIES: Pride