Alexander Disease Mutations Produce Cells with Coexpression of Glial Fibrillary Acidic Protein and NG2 in Neurosphere Cultures and Inhibit Differentiation into Mature Oligodendrocytes.

ABSTRACT:

Background

Alexander disease (AxD) is a rare disease caused by mutations in the gene encoding glial fibrillary acidic protein (GFAP). The disease is characterized by presence of GFAP aggregates in the cytoplasm of astrocytes and loss of myelin.

Objectives

Determine the effect of AxD-related mutations on adult neurogenesis.

Methods

We transfected different types of mutant GFAP into neurospheres using the nucleofection technique.

Results

We find that mutations may cause coexpression of GFAP and NG2 in neurosphere cultures, which would inhibit the differentiation of precursors into oligodendrocytes and thus explain the myelin loss occurring in the disease. Transfection produces cells that differentiate into new cells marked simultaneously by GFAP and NG2 and whose

SUBMITTER: Gomez-Pinedo U 

PROVIDER: S-EPMC5459916 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature