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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Mlh1tm1Rak
targeted mutation 1, Raju Kucherlapati
MGI:1858054
Summary 9 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Mlh1tm1Rak/Mlh1tm1Rak involves: 129P2/OlaHsd MGI:3783420
hm2
Mlh1tm1Rak/Mlh1tm1Rak involves: 129P2/OlaHsd * C57BL/6 MGI:2663863
hm3
Mlh1tm1Rak/Mlh1tm1Rak involves: 129P2/OlaHsd * C57BL/6 * C57BL/6J MGI:4366921
ht4
Mlh1tm1Rak/Mlh1+ involves: 129P2/OlaHsd * C57BL/6 MGI:4412023
cx5
Mlh1tm1Rak/Mlh1tm1Rak
Poletm1.1Bdp/Poletm1.1Bdp
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6 * C57BL/6J MGI:4366924
cx6
Mlh1tm1Rak/Mlh1tm1Rak
Pold1tm1.1Bdp/Pold1tm1.1Bdp
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6 * C57BL/6J MGI:4366923
cx7
Fan1em1Mem/Fan1em1Mem
Htttm5Mem/Htt+
Mlh1tm1Rak/Mlh1tm1Rak
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6J MGI:6489857
cx8
Apctm1Rak/Apc+
Mlh1tm1Rak/Mlh1+
involves: 129P2/OlaHsd * C57BL/6 MGI:4412022
cx9
Apctm1Rak/Apc+
Mlh1tm1Rak/Mlh1tm1Rak
involves: 129P2/OlaHsd * C57BL/6 MGI:4412021


Genotype
MGI:3783420
hm1
Allelic
Composition
Mlh1tm1Rak/Mlh1tm1Rak
Genetic
Background
involves: 129P2/OlaHsd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mlh1tm1Rak mutation (1 available); any Mlh1 mutation (42 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 50% of mice die by 30 weeks of age and all mice are dead by 64 weeks of age

neoplasm
• 35 mice exhibit small intestine tumors (19 of 31 are adenocarcinomas and 12 are adenomas)
• 8 of 66 mice exhibited both lymphomas and intestinal tumor
• 4 of 66 mice develop squamous basal cell carcinoma of the skin
• 35 of 66 mice develop T and B cell lymphomas
• 8 of 66 mice exhibited both lymphomas and intestinal tumor

homeostasis/metabolism
• mouse embryonic fibroblasts are resistant to the effects of cisplatin unlike wild-type cells and exhibit a disruption of cell cycle arrest and reduced apoptosis following exposure

cellular
• mouse embryonic fibroblasts are resistant to the effects of cisplatin unlike wild-type cells and exhibit a disruption of cell cycle arrest and reduced apoptosis following exposure

integument
• 4 of 66 mice develop squamous basal cell carcinoma of the skin

digestive/alimentary system
• 35 mice exhibit small intestine tumors (19 of 31 are adenocarcinomas and 12 are adenomas)
• 8 of 66 mice exhibited both lymphomas and intestinal tumor




Genotype
MGI:2663863
hm2
Allelic
Composition
Mlh1tm1Rak/Mlh1tm1Rak
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mlh1tm1Rak mutation (1 available); any Mlh1 mutation (42 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 50% of mice die by 7 months (J:53451)
• early moridity in mice treated with DES (J:98929)
• death at 161-164 days in treated mice (J:98929)
• death at 297-299 days for untreated mice (J:98929)

reproductive system
• oocytes do not complete meiosis II
• arrest at pachytene

neoplasm
• 72% of mice develop tumors
• 33% of mice develop gastrointestinal tumors in the stomach, duodenum, jejunum, ileum, and colon
• 50% of mice develop adenomas
• some mice develop non-Hodgkin's lymphoma (5 of 8 T cell type, 2 of 8 B cell type) and Hodgkin's lymphoma
• 2 of 8 (J:53451)
• five of eight lymphomas tested are B cell lymphomas (J:98929)
• mice develop 13% carcinomas and 37% early invasive carcinomas
• one mouse develops sweat gland carcinoma

cellular
• apoptosis of arrested germ cells

endocrine/exocrine glands

digestive/alimentary system
• 33% of mice develop gastrointestinal tumors in the stomach, duodenum, jejunum, ileum, and colon

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Lynch syndrome DOID:3883 OMIM:PS120435
J:53451




Genotype
MGI:4366921
hm3
Allelic
Composition
Mlh1tm1Rak/Mlh1tm1Rak
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6 * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mlh1tm1Rak mutation (1 available); any Mlh1 mutation (42 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice die before 15 months

neoplasm
• 42% of mice develop intestinal adenomas and adenocarcinomas compared with 5% of wild-type mice
• 42% of mice develop intestinal adenomas and adenocarcinomas compared with 5% of wild-type mice
• 4% of mice develop nodal lymphomas and 31% of mice develop follicular lymphomas
• in 38% of mice unlike wild-type mice

endocrine/exocrine glands
• in 38% of mice unlike wild-type mice

digestive/alimentary system
• 42% of mice develop intestinal adenomas and adenocarcinomas compared with 5% of wild-type mice
• 42% of mice develop intestinal adenomas and adenocarcinomas compared with 5% of wild-type mice




Genotype
MGI:4412023
ht4
Allelic
Composition
Mlh1tm1Rak/Mlh1+
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mlh1tm1Rak mutation (1 available); any Mlh1 mutation (42 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 50% of mice die by 18 months

neoplasm
• 32% of mice develop tumors at an average age of 9.8 months
• 14% of mice develop gastrointestinal tumors in the duodenum, jejunum, and colon
• mice develop an equal number of gastrointestinal tract adenocarcinomas and early invasive carcinoma
• non-Hodgkin's lymphoma in one mouse
• mice develop an equal number of gastrointestinal tract adenocarcinomas and early invasive carcinoma
• mice develop sweat gland carcinoma
• mice develop an equal number of gastrointestinal tract adenocarcinomas and early invasive carcinoma
• one mouse develops lung bronchio-alveolar carcinoma
• in the cervix of two mice

digestive/alimentary system
• 14% of mice develop gastrointestinal tumors in the duodenum, jejunum, and colon
• mice develop an equal number of gastrointestinal tract adenocarcinomas and early invasive carcinoma

respiratory system
• one mouse develops lung bronchio-alveolar carcinoma

endocrine/exocrine glands




Genotype
MGI:4366924
cx5
Allelic
Composition
Mlh1tm1Rak/Mlh1tm1Rak
Poletm1.1Bdp/Poletm1.1Bdp
Genetic
Background
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6 * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mlh1tm1Rak mutation (1 available); any Mlh1 mutation (42 available)
Poletm1.1Bdp mutation (0 available); any Pole mutation (99 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice are present in normal ratios up to E14.5; none are recovered at weaning




Genotype
MGI:4366923
cx6
Allelic
Composition
Mlh1tm1Rak/Mlh1tm1Rak
Pold1tm1.1Bdp/Pold1tm1.1Bdp
Genetic
Background
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6 * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mlh1tm1Rak mutation (1 available); any Mlh1 mutation (42 available)
Pold1tm1.1Bdp mutation (0 available); any Pold1 mutation (62 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging




Genotype
MGI:6489857
cx7
Allelic
Composition
Fan1em1Mem/Fan1em1Mem
Htttm5Mem/Htt+
Mlh1tm1Rak/Mlh1tm1Rak
Genetic
Background
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fan1em1Mem mutation (0 available); any Fan1 mutation (98 available)
Htttm5Mem mutation (2 available); any Htt mutation (179 available)
Mlh1tm1Rak mutation (1 available); any Mlh1 mutation (42 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cellular
N
• Htt CAG repeat stability is normal




Genotype
MGI:4412022
cx8
Allelic
Composition
Apctm1Rak/Apc+
Mlh1tm1Rak/Mlh1+
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Apctm1Rak mutation (1 available); any Apc mutation (154 available)
Mlh1tm1Rak mutation (1 available); any Mlh1 mutation (42 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• all mice die by 14 months of age

neoplasm
• 85% of mice develop tumors at an average age of 9.4 months
• 77% of mice develop gastrointestinal tract tumors and 23% of mice develop extra-gastrointestinal tract tumors
• 77% of mice develop gastrointestinal tract tumors
• mice exhibit a 7-fold increase in gastrointestinal tumors compared to Mlh1tm1Rak heterozygotes

digestive/alimentary system
• mice exhibit a 7-fold increase in gastrointestinal tumors compared to Mlh1tm1Rak heterozygotes
• 77% of mice develop gastrointestinal tract tumors




Genotype
MGI:4412021
cx9
Allelic
Composition
Apctm1Rak/Apc+
Mlh1tm1Rak/Mlh1tm1Rak
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Apctm1Rak mutation (1 available); any Apc mutation (154 available)
Mlh1tm1Rak mutation (1 available); any Mlh1 mutation (42 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• all mice die by 4 months of age

neoplasm
• all mice develop tumors at an average age of 3.3 months
• all mice develop gastrointestinal tract tumors and 9% develop non-gastrointestinal tract tumors
• all mice develop gastrointestinal tract tumors
• mice exhibit a 30-fold increase in gastrointestinal tumors compared to Mlh1tm1Rak homozygotes
• mice exhibit a 4- to 5-fold increase in stomach and colon tumors, and a 25- to 100-fold increase in duodenum, jejunum, and ileum compared to in homozygous mice
• one mouse develops skin carcinoma with lung metastasis
• 34% of mice develop microadenomas and 36% adenomas
• non-Hodgkin's lymphoma in two mice
• mice develop gastrointestinal adenocarcinomas, early invasive carcinomas (in 13% of mice), and carcinomas (in 17% of mice)

digestive/alimentary system
• all mice develop gastrointestinal tract tumors
• mice exhibit a 30-fold increase in gastrointestinal tumors compared to Mlh1tm1Rak homozygotes
• mice exhibit a 4- to 5-fold increase in stomach and colon tumors, and a 25- to 100-fold increase in duodenum, jejunum, and ileum compared to in homozygous mice
• at 2 to 4 weeks of age

integument
• one mouse develops skin carcinoma with lung metastasis

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Lynch syndrome DOID:3883 OMIM:PS120435
J:53451





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last database update
03/19/2024
MGI 6.23
The Jackson Laboratory