Phenotypes Associated with This Genotype

Genotype
MGI:3850388

• Allelic Composition: Nf2^tm2Gth/Nf2^tm2Gth; Tg(Mpz-cre)1Brn/0
• Genetic Background: involves: 129P2/OlaHsd * FVB/N

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

mortality/aging

decreased survivor rate ( J:63264 )

premature death ( J:63264 )

• mice have a short lifespan dying by 10 months of age

postnatal lethality, incomplete penetrance ( J:63264 )

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

• however, postnatal lethality can be partially rescued by supplementing food with porridge

reproductive system

infertility ( J:63264 )

neoplasm

increased tumor incidence ( J:63264 )

• after 10 months, mice develop benign and malignant Schwann cell tumors

craniofacial

delayed tooth eruption ( J:63264 )

• at P17 and P19, molar eruption is retarded or absent

failure of tooth eruption ( J:63264 )

• at P17 and P19, molar eruption is retarded or absent

nervous system

abnormal Schwann cell morphology ( J:63264 )

• 2 of 4 mice and 4 months and 1 mouse at P17 exhibit hyperplasia or hypertrophy in Schwann cells of distal peripheral nerves

• at P19 and P33, Schwann cells are without a clear relation with an axon and many of the myelin sheaths herniated and loop into the central axonal region unlike in wild-type mice

growth/size/body

delayed tooth eruption ( J:63264 )

• at P17 and P19, molar eruption is retarded or absent

failure of tooth eruption ( J:63264 )

• at P17 and P19, molar eruption is 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 )

• at P17 and P19, molar eruption is retarded or absent

failure of tooth eruption ( J:63264 )

• at P17 and P19, molar eruption is retarded or absent

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