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 |