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Increased IRP1 activity in Friedreich ataxia

Authors
Journal
Gene
0378-1119
Publisher
Elsevier
Publication Date
Volume
354
Identifiers
DOI: 10.1016/j.gene.2005.04.040
Keywords
  • Iron–Sulfur Cluster
  • Mitochondria
  • Rna Binding
Disciplines
  • Biology

Abstract

Abstract In low-iron conditions, the cytosolic iron-regulatory protein IRP1 binds to iron-responsive elements (IREs) in mRNAs encoding iron-regulated proteins. In high-iron conditions, IRP1 incorporates an iron–sulfur cluster (ISC), which interferes with IRE binding and prevents intracellular iron accumulation. Here we demonstrate an incomplete shift of IRP1 to its ISC form in Friedreich ataxia (FRDA) fibroblasts, associated with decreased activities of ISC respiratory complexes. Our data suggest an impaired adaptive response to iron accumulation in FRDA cells.

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