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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Tg(Mpz-cre)2Brn
transgene insertion 2, Anton Berns
MGI:3047001
Summary 4 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
 		Nf2tm2Gth/Nf2tm2Gth
Trp53tm1Brd/Trp53+
Tg(Mpz-cre)2Brn/0 		involves: 129P2/OlaHsd * 129S7/SvEvBrd * FVB/N MGI:3850407
cn2
 		Nf2tm2Gth/Nf2tm2Gth
Trp53tm1Brd/Trp53tm1Brd
Tg(Mpz-cre)2Brn/0 		involves: 129P2/OlaHsd * 129S7/SvEvBrd * FVB/N MGI:3850408
cn3
 		Nf2tm2Gth/Nf2tm2Gth
Tg(Mpz-cre)2Brn/0 		involves: 129P2/OlaHsd * FVB/N MGI:3850389
cn4
 		Nf2tm1Gth/Nf2tm2Gth
Tg(Mpz-cre)2Brn/0 		involves: 129P2/OlaHsd * FVB/N MGI:3850394


Genotype
MGI:3850407
cn1
Allelic
Composition		 Nf2tm2Gth/Nf2tm2Gth
Trp53tm1Brd/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:
Nf2tm2Gth mutation (3 available); any Nf2 mutation (67 available)
Tg(Mpz-cre)2Brn mutation (1 available)
Trp53tm1Brd mutation (5 available); any Trp53 mutation (238 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
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
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
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

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

cellular
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




Genotype
MGI:3850408
cn2
Allelic
Composition		 Nf2tm2Gth/Nf2tm2Gth
Trp53tm1Brd/Trp53tm1Brd
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:
Nf2tm2Gth mutation (3 available); any Nf2 mutation (67 available)
Tg(Mpz-cre)2Brn mutation (1 available)
Trp53tm1Brd mutation (5 available); any Trp53 mutation (238 available)
♀ 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 sarcoma incidence ( J:92413 )
• at 2 to 3.5 months, 25% of mice develop 4 sarcoma mainly in the ganglia
increased neurofibrosarcoma incidence ( J:92413 )
• at 2 to 3.5 months, 50% of mice develop 4 malignant peripheral nerve sheath tumors
increased osteoma incidence ( J:92413 )
• one mouse developed osteomas

skeleton
increased osteoma incidence ( J:92413 )
• one mouse developed osteomas
abnormal osteoblast morphology ( J:92413 )
• 25% of mice develop osteogenic hyperplasia

nervous system
abnormal Schwann cell morphology ( J:92413 )
• 38% of mice exhibit Schwann cell hyperplasia
increased neurofibrosarcoma incidence ( J:92413 )
• at 2 to 3.5 months, 50% of mice develop 4 malignant peripheral nerve sheath tumors

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

cellular
abnormal Schwann cell morphology ( J:92413 )
• 38% of mice exhibit Schwann cell hyperplasia




Genotype
MGI:3850389
cn3
Allelic
Composition		 Nf2tm2Gth/Nf2tm2Gth
Tg(Mpz-cre)2Brn/0
Genetic
Background		 involves: 129P2/OlaHsd * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nf2tm2Gth mutation (3 available); any Nf2 mutation (67 available)
Tg(Mpz-cre)2Brn mutation (1 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
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
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)
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)

neoplasm
osseous metaplasia ( J:92413 )
• 25% of mice develop osseous metaplasia
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 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

immune system

craniofacial
abnormal tooth development ( J:92413 )
• one mouse exhibited odontoblastic hyperplasia

respiratory system

cellular
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)

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




Genotype
MGI:3850394
cn4
Allelic
Composition		 Nf2tm1Gth/Nf2tm2Gth
Tg(Mpz-cre)2Brn/0
Genetic
Background		 involves: 129P2/OlaHsd * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nf2tm1Gth mutation (1 available); any Nf2 mutation (67 available)
Nf2tm2Gth mutation (3 available); any Nf2 mutation (67 available)
Tg(Mpz-cre)2Brn mutation (1 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
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

immune system

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




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09/30/2025
MGI 6.24 		The Jackson Laboratory