Table 2.

Mouse lines with mutations in MMR genes

Genotype	Tumour		MSI incidencea		Fertility Male/female	Reference
	Spectrumb	Incidence	Mononucleotide	Dinucleotide
MutS homologues
MSH2−/−	Lymphoma, GI, skin and other tumours	High	High	High	+/+	de Wind et al. (1995), Reitmair et al. (1995, 1997), Smits et al. (2000)
MSH2 C674A/C674A	Lymphoma, GI, skin	High	High	High	+/+	Lin et al. (2004)
MSH3−/−	GI tumours	Low	Moderate	High	+/+	Edelmann et al. (2000), de Wind et al. (1999)
MSH6−/−	Lymphoma, GI, skin and	High	None	Low	+/+	Edelmann et al. (1997)
MSH6 TI217D/T1217D	Lymphoma, GI, skin	High	High	High	+/+	Yang et al. (2004a,b)
MSH3−/− MSH6−/−	Lymphoma, GI, skin and other tumours	High	High	High	+/+	Edelmann et al. (2000), de Wind et al. (1999)
MSH4−/−	None	None	N/A	N/A	−/−	Kneitz et al. (2000)
MSH5−/−	None	None	N/A	N/A	−/−	Edelmann et al. (1999a), de Vries et al. (1999)
MutL homologues
MLH1−/−	Lymphoma, GI, skin and other tumours	High	High	High	−/−	Baker et al. (1996), Edelmann et al. (1996), Prolla et al. (1998), Edelmann et al. (1999b)
PMS1−/−	None	None	Low	Low	+/+	Prolla et al. (1998)
PMS2−/−	Lymphoma and sarcoma	High	High	High	−/+	Baker et al. (1995), Prolla et al. (1998)
MLH3−/−	N/A	N/A	Low	Low	−/−;	Lipkin et al. (2002)
Exonuclease
EXO1−/−	Lymphoma	Moderate	High	Low	−/−	Wei et al. (2003)

GI tumours: Adenoma, adenocarcinoma, and flat adenoma in gastrointestinal organs. Skin tumours: Squamous cell carcinoma, keratoacanthoma, sebaceous (adenoma, epithelioma, carcinoma), papilloma, haemangioma, and pylomatricoma. Other tumours include: Tumours of the uterus, brain, lung, liver, and mammary gland. Sarcoma: Leiomyosarcoma, myxoid sarcoma, and fibroid sarcoma. Adapted from Edelmann and Edelmann (2004) and used with permission.

^aMSI was analysed in tumour samples, normal tissue or culture cells.

^bTumour spectrum includes: Lymphoma: B and T cell lymphoma (NHL, non-Hodgkin’s lymphoma).