Altered Ca2+ responses and antioxidant properties in Friedreich's ataxia-like cerebellar astrocytes.

ABSTRACT: Friedreich's ataxia (FRDA) is a neurodegenerative disorder characterized by severe neurological signs, affecting the peripheral and central nervous system, caused by reduced frataxin protein (FXN) levels. Although several studies have highlighted cellular dysfunctions in neurons, there is limited information on the effects of FXN depletion in astrocytes and on the potential non-cell autonomous mechanisms affecting neurons in FRDA. In this study, we generated a model of FRDA cerebellar astrocytes to unveil phenotypic alterations that might contribute to cerebellar atrophy. We treated primary cerebellar astrocytes with an RNA interference-based approach, to achieve a reduction of FXN comparable to that observed in individuals with FRDA. These FRDA-like astrocytes display some typical feature

SUBMITTER: Marullo C 

PROVIDER: S-EPMC11828468 | biostudies-literature | 2025 Jan

REPOSITORIES: biostudies-literature