Edaravone and mitochondrial transfer as potential therapeutics for vanishing white matter disease astrocyte dysfunction.

ABSTRACT:

Introduction

Previous research has suggested that vanishing white matter disease (VWMD) astrocytes fail to fully differentiate and respond differently to cellular stresses compared to healthy astrocytes. However, few studies have investigated potential VWMD therapeutics in monoculture patient-derived cell-based models.

Methods

To investigate the impact of alterations in astrocyte expression and function in VWMD, astrocytes were differentiated from patient and control induced pluripotent stem cells and analyzed by proteomics, pathway analysis, and functional assays, in the absence and presence of stressors or potential therapeutics.

Results

Vanishing white matter disease astrocytes demonstrated significantly reduced expression of astrocyte markers and markers of inflam

SUBMITTER: Ng NS 

PROVIDER: S-EPMC10401142 | biostudies-literature | 2023 Sep

REPOSITORIES: biostudies-literature