Sex Is a major determinant of neuronal dysfunction in neurofibromatosis type 1.

ABSTRACT:

Objective

Children with neurofibromatosis-1 (NF1) are at risk for developing numerous nervous system abnormalities, including cognitive problems and brain tumors (optic pathway glioma). Currently, there are few prognostic factors that predict clinical manifestations or outcomes in patients, even in families with an identical NF1 gene mutation. In this study, we leveraged Nf1 genetically engineered mice (GEM) to define the potential role of sex as a clinically relevant modifier of NF1-associated neuronal dysfunction.

Methods

Deidentified clinical data were analyzed to determine the impact of sex on optic glioma-associated visual decline in children with NF1. In addition, Nf1 GEM were employed as experimental platforms to investigate sexually dimorphic differences in learning/

SUBMITTER: Diggs-Andrews KA 

PROVIDER: S-EPMC4172335 | biostudies-literature | 2014 Feb

REPOSITORIES: biostudies-literature