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pubmed-article:17262846pubmed:abstractTextFriedreich'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 test whether the expanded GAA triplet-repeat sequence undergoes further expansion in DRGs as a possible mechanism underlying the progressive pathology seen in patients.lld:pubmed
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pubmed-article:17262846pubmed:articleTitleProgressive GAA expansions in dorsal root ganglia of Friedreich's ataxia patients.lld:pubmed
pubmed-article:17262846pubmed:affiliationDepartment of Biochemistry and Molecular Biology, University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA.lld:pubmed
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