Temporal Phenotypic Changes in Huntington's Disease Models for Preclinical Studies.

ABSTRACT:

Background

Mouse models bearing genetic disease mutations are instrumental in the development of therapies for genetic disorders. Huntington's disease (HD) is a late-onset lethal dominant genetic disorder due to a CAG repeat within exon 1 of the Huntingtin (Htt) gene. Several mice were developed to model HD through the expression of a transgenic fragment (exon 1 of the human HTT), the knock-in mutation of the CAG repeat in the context of the mouse Htt gene, or the full-length HTT human gene. The different mouse models present distinct onset, symptoms, and progression of the disease.

Objective

The objective of this study is to advise on the best behavioral tests to assess disease progression in three HD mouse models.

Methods

We tested N171-82Q transgenic mice, zQ175 kn

SUBMITTER: St-Cyr S 

PROVIDER: S-EPMC9028736 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature