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Article

Progressive gaa expansions in dorsal root ganglia of Friedreich's ataxia patients

1 Jan 2007

Abstract excerpt

OBJECTIVE: Friedreich's ataxia patients are homozygous for expanded alleles of a GAA triplet-repeat sequence in the FXN gene. Patients develop progressive ataxia due to primary neurodegeneration involving the dorsal root ganglia (DRGs). The selective neurodegeneration is due to the sensitivity of DRGs to frataxin deficiency; however, the progressive nature of the disease remains unexplained. Our objective was to...

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PMID: 17262846
DOI: 10.1002/ana.21052

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