Table 2.

Effect of mutations on recruitment of Mlh1 or MutL.

Mutation	ScMlh1 residue	EcMutL residue	hMLH1 residue	Recruitment defect	Notes	Reference
EcMutL K52C	K54	K52	K57	Yes	Connector domain interface	[21]
ScMlh1 K54C	K54	K52	K57	Yes	Connector domain interface	This study
EcMutL R55D, R57D	Q57, T59	R55, R57	Q60, Q62	Yes	ATPase/core domain interface	[21]
ScMlh1 Q57A, T59A	Q57, T59	R55, R57	Q60, Q62	Yes	ATPase/core domain interface	This study
EcMutL R55D, R57D, A138E, H139A	Q57, T59, A140, G141	R55, R57, A138, H139	Q60, Q62, A143, G144	Yes	Both interfaces	[21]
hMLH1 N64S	N61	N59	N64	No	Next to ATPase/core domain interface; Lynch syndrome	[26]
hMLH1 H112A	R109	R107	H112	Yes	Connector domain interface	[26]
hMLH1 H112D	R109	R107	H112	Yes	Connector domain interface	[26]
hMLH1 T117M	T114	S112	T117	No	Mostly buried; Lynch syndrome	[26]
hMLH1 R127E	R124	Q122	R127	Yes	Connector domain interface	[26]
hMLH1 A128P	V125	A123	A128	Yes	Connector domain interface	[26]
hMLH1 Y130A	Y127	A125	Y130	Yes	Connector domain interface	[26]
hMLH1 D132H	E129	G127	D132	No	Loop between connector domain interface residues; disordered in co-crystal structure	[26]
EcMutL A138E	A140	A138	A143	Yes	ATPase/core domain interface	[21]
EcMutL A138E, H139A	A140, G141	A138, H139	A143, G144	Yes	ATPase/core domain interface	[21]
ScMlh1 A140E, G141A	A140, G141	A138, H139	A143, G144	n.d.	ATPase/core domain interface; MMR defective in both ScMLH1 and ScPMS1	This study
hMLH1 R265C	R265	R261	R265	No	Opposite face of molecule; Lynch syndrome	[26]