Striatal Mutant Huntingtin Protein Levels Decline with Age in Homozygous Huntington's Disease Knock-In Mouse Models.

ABSTRACT:

Background

Huntington's disease (HD) is a progressive neurodegenerative disorder associated with aging, caused by an expanded polyglutamine (polyQ) repeat within the Huntingtin (HTT) protein. In HD, degeneration of the striatum and atrophy of the cortex are observed while cerebellum is less affected.

Objective

To test the hypothesis that HTT protein levels decline with age, which together with HTT mutation could influence disease progression.

Methods

Using whole brain cell lysates, a unique method of SDS-PAGE and western analysis was used to quantitate HTT protein, which resolves as a monomer and as a high molecular weight species that is modulated by the presence of transglutaminase 2. HTT levels were measured in striatum, cortex and cerebellum in congenic homozygous

SUBMITTER: Franich NR 

PROVIDER: S-EPMC6002862 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature