All Phenotypes Tg(Mpz-cre)2Brn MGI Mouse

Phenotypes associated with this allele

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Nf2^tm2Gth/Nf2^tm2Gth Trp53^tm1Brd/Trp53⁺ Tg(Mpz-cre)2Brn/0	involves: 129P2/OlaHsd * 129S7/SvEvBrd * FVB/N	MGI:3850407
cn2	Nf2^tm2Gth/Nf2^tm2Gth Trp53^tm1Brd/Trp53^tm1Brd Tg(Mpz-cre)2Brn/0	involves: 129P2/OlaHsd * 129S7/SvEvBrd * FVB/N	MGI:3850408
cn3	Nf2^tm2Gth/Nf2^tm2Gth Tg(Mpz-cre)2Brn/0	involves: 129P2/OlaHsd * FVB/N	MGI:3850389
cn4	Nf2^tm1Gth/Nf2^tm2Gth Tg(Mpz-cre)2Brn/0	involves: 129P2/OlaHsd * FVB/N	MGI:3850394

Allelic Composition	Nf2^tm2Gth/Nf2^tm2Gth Trp53^tm1Brd/Trp53⁺ Tg(Mpz-cre)2Brn/0
Genetic Background	involves: 129P2/OlaHsd * 129S7/SvEvBrd * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nf2^tm2Gth mutation (3 available); any Nf2 mutation (65 available)
Tg(Mpz-cre)2Brn mutation (1 available)
Trp53^tm1Brd mutation (5 available); any Trp53 mutation (232 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

decreased survivor rate ( J:92413 )

premature death ( J:92413 )

• median age of death is 4.5 months

neoplasm

osseous metaplasia ( J:92413 )

• 23% of mice develop osseous metaplasia

increased sarcoma incidence ( J:92413 )

• one mouse developed lymphosarcoma

increased leiomyosarcoma incidence ( J:92413 )

• in 8% of mice with metastasis to the liver

increased nervous system tumor incidence ( J:92413 )

• 85% of mice develop peripheral nerve tumors

increased neurofibroma incidence ( J:92413 )

• 8% of mice develop neurofibroma

increased neurofibrosarcoma incidence ( J:92413 )

• 77% of mice develop peripheral nerve tumors from peripheral nerves of the limbs and from the dorsal root ganglia

increased Schwannoma incidence ( J:92413 )

• 11% of mice develop schwannoma

skeleton

abnormal tooth development ( J:92413 )

• 35% of mice exhibit odontoblastic hyperplasia

abnormal osteoblast morphology ( J:92413 )

• 15% of mice develop osteogenic hyperplasia

nervous system

increased nervous system tumor incidence ( J:92413 )

• 85% of mice develop peripheral nerve tumors

increased neurofibroma incidence ( J:92413 )

• 8% of mice develop neurofibroma

increased neurofibrosarcoma incidence ( J:92413 )

• 77% of mice develop peripheral nerve tumors from peripheral nerves of the limbs and from the dorsal root ganglia

increased Schwannoma incidence ( J:92413 )

• 11% of mice develop schwannoma

abnormal Schwann cell morphology ( J:92413 )

• 81% of mice exhibit Schwann cell hyperplasia with increased incidence of diffuse Schwann cell hyperplasia in major peripheral nerve trunks

renal/urinary system

abnormal renal tubule morphology ( J:92413 )

• 73% of mice exhibit renal tubular cell hyperplasia

vision/eye

cataract ( J:92413 )

• 85% of mice

craniofacial

abnormal tooth development ( J:92413 )

• 35% of mice exhibit odontoblastic hyperplasia

growth/size/body

abnormal tooth development ( J:92413 )

• 35% of mice exhibit odontoblastic hyperplasia

muscle

increased leiomyosarcoma incidence ( J:92413 )

• in 8% of mice with metastasis to the liver

Allelic Composition	Nf2^tm2Gth/Nf2^tm2Gth Trp53^tm1Brd/Trp53^tm1Brd Tg(Mpz-cre)2Brn/0
Genetic Background	involves: 129P2/OlaHsd * 129S7/SvEvBrd * FVB/N

Find Mice

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

premature death ( J:92413 )

• mice die a few months of birth

neoplasm

osseous metaplasia ( J:92413 )

• 38% of mice develop osseous metaplasia

increased tumor incidence ( J:92413 )

increased sarcoma incidence ( J:92413 )

• at 2 to 3.5 months, 25% of mice develop 4 sarcoma mainly in the ganglia

increased rhabdomyosarcoma incidence ( J:92413 )

• in one mouse

increased neurofibrosarcoma incidence ( J:92413 )

• at 2 to 3.5 months, 50% of mice develop 4 malignant peripheral nerve sheath tumors

increased extremity angiosarcoma incidence ( J:92413 )

• in 63% of mice

increased osteosarcoma incidence ( J:92413 )

• in one mouse

increased osteoma incidence ( J:92413 )

• one mouse developed osteomas

skeleton

increased osteosarcoma incidence ( J:92413 )

• in one mouse

increased osteoma incidence ( J:92413 )

• one mouse developed osteomas

abnormal osteoblast morphology ( J:92413 )

• 25% of mice develop osteogenic hyperplasia

nervous system

increased neurofibrosarcoma incidence ( J:92413 )

• at 2 to 3.5 months, 50% of mice develop 4 malignant peripheral nerve sheath tumors

abnormal Schwann cell morphology ( J:92413 )

• 38% of mice exhibit Schwann cell hyperplasia

renal/urinary system

abnormal renal tubule morphology ( J:92413 )

• 63% of mice exhibit renal tubular cell hyperplasia

vision/eye

cataract ( J:92413 )

• 75% of mice

muscle

increased rhabdomyosarcoma incidence ( J:92413 )

• in one mouse

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

decreased survivor rate ( J:63264 )

premature death ( J:63264 )

• few mice survive beyond 24 months of age

postnatal lethality, incomplete penetrance ( J:63264 )

• fewer mice than expected are obtained (no time point for lethality given)

growth/size/body

abnormal tooth development ( J:92413 )

• one mouse exhibited odontoblastic hyperplasia

decreased body size ( J:63264 )

• although not as much as in Tg(P0-Cre)1Gth conditional knock-outs of Nf2

decreased body weight ( J:63264 )

• although not as much as in Tg(P0-Cre)1Gth conditional knock-outs of Nf2

nervous system

increased neurofibrosarcoma incidence ( J:92413 )

• 1 of 27 mice develop peripheral nerve tumors

increased Schwannoma incidence ( J:63264 , J:92413 )

• 4% of mice develop schwannomas (J:63264)

abnormal Schwann cell morphology ( J:63264 , J:92413 )

• mice exhibit Schwann cell hyperplasia (J:63264)

• occasionally whorls of thin cytoplasmic processes are observed in Schwann cells (J:63264)

• 100% of mice exhibit Schwann cell hyperplasia (J:92413)

neoplasm

osseous metaplasia ( J:92413 )

• 25% of mice develop osseous metaplasia

increased lung adenocarcinoma incidence ( J:92413 )

• in one mouse

increased renal carcinoma incidence ( J:92413 )

• 25% of mice develop carcinoma in situ in the kidney

increased neurofibrosarcoma incidence ( J:92413 )

• 1 of 27 mice develop peripheral nerve tumors

increased extremity angiosarcoma incidence ( J:92413 )

• in one mouse

increased Schwannoma incidence ( J:63264 , J:92413 )

• 4% of mice develop schwannomas (J:63264)

skeleton

abnormal tooth development ( J:92413 )

• one mouse exhibited odontoblastic hyperplasia

abnormal osteoblast morphology ( J:92413 )

• 25% of mice develop osteogenic hyperplasia

renal/urinary system

increased renal carcinoma incidence ( J:92413 )

• 25% of mice develop carcinoma in situ in the kidney

abnormal renal tubule morphology ( J:92413 )

• 25% of mice develop carcinoma in situ in the kidney

vision/eye

cataract ( J:63264 , J:92413 )

• in 59% of mice, in which 50% of cases are bilateral (J:63264)

• 86% of mice (J:92413)

hearing/vestibular/ear

increased susceptibility to otitis media ( J:63264 )

immune system

increased susceptibility to otitis media ( J:63264 )

craniofacial

abnormal tooth development ( J:92413 )

• one mouse exhibited odontoblastic hyperplasia

respiratory system

increased lung adenocarcinoma incidence ( J:92413 )

• in one mouse

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
otitis media		DOID:10754		J:63264

Allelic Composition	Nf2^tm1Gth/Nf2^tm2Gth Tg(Mpz-cre)2Brn/0
Genetic Background	involves: 129P2/OlaHsd * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nf2^tm1Gth mutation (1 available); any Nf2 mutation (65 available)
Nf2^tm2Gth mutation (3 available); any Nf2 mutation (65 available)
Tg(Mpz-cre)2Brn mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

decreased survivor rate ( J:63264 )

premature death ( J:63264 )

• 50% of mice die within the first year

craniofacial

delayed tooth eruption ( J:63264 )

• retarded or absent

failure of tooth eruption ( J:63264 )

• retarded or absent

growth/size/body

delayed tooth eruption ( J:63264 )

• retarded or absent

failure of tooth eruption ( J:63264 )

• retarded or absent

decreased body size ( J:63264 )

decreased body weight ( J:63264 )

hearing/vestibular/ear

increased susceptibility to otitis media ( J:63264 )

immune system

increased susceptibility to otitis media ( J:63264 )

skeleton

delayed tooth eruption ( J:63264 )

• retarded or absent

failure of tooth eruption ( J:63264 )

• retarded or absent

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
otitis media		DOID:10754		J:63264

Contributing Projects: Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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