Iron-Sulfur Clusters and Iron Responsive Element Binding Proteins Mediate Iron Accumulation in Corneal Endothelial Cells in Fuchs Dystrophy.

ABSTRACT:

Purpose

Evidence suggests that corneal endothelial cell (CEC) death in Fuchs endothelial corneal dystrophy (FECD) is due to ferroptosis, an iron-mediated cell death. Iron-sulfur cluster (ISC)-containing aconitases and the iron responsive element binding proteins IREBP1 and IREBP2 are known mediators of iron homeostasis. This study investigates mechanisms underlying iron dysregulation in CECs and proposes a role for ISCs and IREBPs in the context of FECD pathogenesis.

Methods

We studied gene expression of proteins responsible for ISC synthesis and iron homeostasis in human and mouse CECs and analyzed published RNA sequencing datasets. We validated a subset of transcriptional changes between FECD and control tissues using microfluidic Western blotting with human CEC tissues. F

SUBMITTER: Hartness EM 

PROVIDER: S-EPMC11993131 | biostudies-literature | 2025 Apr

REPOSITORIES: biostudies-literature