The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis.

doi:10.1093/hmg/8.3.425

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Article

The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis.

Human molecular genetics - 1 Mar 1999

Wong A, Yang J, Cavadini P, Gellera C, Lonnerdal B, Taroni F, Cortopassi G

Abstract excerpt

Expansions of an intronic GAA repeat reduce the expression of frataxin and cause Friedreich's ataxia (FRDA), an autosomal recessive neurodegenerative disease. Frataxin is a mitochondrial protein, and disruption of a frataxin homolog in yeast results in increased sensitivity to oxidant stress, inc...

Topics

Apoptosis
Base Sequence
Calcium
Case-Control Studies
Caspase 3
Caspases
Cell Line
Chelating Agents
DNA Primers
Deferoxamine
Egtazic Acid
Fibroblasts

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Friedreich Ataxia

Humans

Hydrogen Peroxide

Iron

Iron-Binding Proteins

Mutation

Source

Open in PubMed

Identifiers

PMID: 9949201
DOI: 10.1093/hmg/8.3.425

Genes

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 30 Aug 2026. Prepared record publication-9949201-2026-08-30.1. Manifest publication-graph-cohort-2026-08-30.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 30 Aug 2026.

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