Insulin-like growth factor-2 does not improve behavioral deficits in mouse and rat models of Angelman Syndrome.

ABSTRACT:

Background

Angelman Syndrome (AS) is a rare neurodevelopmental disorder for which there is currently no cure or effective therapeutic. Since the genetic cause of AS is known to be dysfunctional expression of the maternal allele of ubiquitin protein ligase E3A (UBE3A), several genetic animal models of AS have been developed. Both the Ube3a maternal deletion mouse and rat models of AS reliably demonstrate behavioral phenotypes of relevance to AS and therefore offer suitable in vivo systems in which to test potential therapeutics. One promising candidate treatment is insulin-like growth factor-2 (IGF-2), which has recently been shown to ameliorate behavioral deficits in the mouse model of AS and improve cognitive abilities across model systems.

Methods

We used both the Ube3a ma

SUBMITTER: Berg EL 

PROVIDER: S-EPMC8444390 | biostudies-literature | 2021 Sep

REPOSITORIES: biostudies-literature