Table 1.

The role of MutS and MutL proteins in somatic instability in mouse models of the FXDs, FRDA and HD

Protein	Effect	FXDs	FRDA	HD
MutS	MSH2	↑	[66]	[65]	[105, 106]
	MSH3	↑	[68]		[62, 108]
	MSH6	↑	[67]	[65]
		–			[62]
MutL	MLH1	↑		[69]	[107]
	MLH3	↑	[59]		[107]
	PMS1	↑	[70]
	PMS2	↑	[70]
		↓		[65]

↑: promotes expansion. ↓: prevents expansion; –: no effect.