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. 2014 Jun 27;9(6):e100523. doi: 10.1371/journal.pone.0100523

Table 3. Effect of MutLα-heterodimers on GAA repeat expansion dynamics and FXN transcription levels.

Intergenerational GAA repeat expansions Somatic GAA repeat expansions Brain and cerebellum FXN transcription level
Pms2 deficiency Increased Increased Decreased
Mlh1 deficiency Decreased Decreased Decreased