Phenotypes Associated with This Genotype

Genotype
MGI:3850407

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

• ♀: 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

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