Phenotypes Associated with This Genotype

Genotype
MGI:3834667

• Allelic Composition: Tg(KRT5-tTA)1216Glk/0; Tg(tetO-LMNA*G608G,-EGFP)VF1-07Maer/0
• Genetic Background: involves: FVB/N

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

mortality/aging

premature death ( J:145312 )

• median survival is 14 weeks for mice withdrawn from doxycycline at 21 days and 7 weeks for those withdrawn at P0

• median survival can be increased from 7 weeks to 29 by providing mice with soft pellets on the cage floor

skeleton

abnormal lower incisor morphology ( J:145312 )

• following doxycycline withdrawal at birth, mice exhibit changes in the lower incisors and surrounding tissue unlike in wild-type mice

craniofacial

abnormal mouth morphology ( J:145312 )

• following doxycycline withdrawal at birth, mice exhibit increased impaction of food or bedding material with acute inflammation to overt necrosis of the pulp, accumulation of cellulose in the pulp, and spread of inflammation into the periodontal ligament and surrounding bony structure unlike in wild-type mice

abnormal lower incisor morphology ( J:145312 )

• following doxycycline withdrawal at birth, mice exhibit changes in the lower incisors and surrounding tissue unlike in wild-type mice

digestive/alimentary system

stomach hypoplasia ( J:145312 )

• following doxycycline withdrawal, mice exhibit hypoplasia in the stomach unlike in wild-type mice

growth/size/body

abnormal mouth morphology ( J:145312 )

• following doxycycline withdrawal at birth, mice exhibit increased impaction of food or bedding material with acute inflammation to overt necrosis of the pulp, accumulation of cellulose in the pulp, and spread of inflammation into the periodontal ligament and surrounding bony structure unlike in wild-type mice

abnormal lower incisor morphology ( J:145312 )

• following doxycycline withdrawal at birth, mice exhibit changes in the lower incisors and surrounding tissue unlike in wild-type mice

decreased body weight ( J:145312 )

• at 7 weeks of age in mice withdrawn from doxycycline at birth

endocrine/exocrine glands

abnormal sebocyte differentiation ( J:145312 )

• six weeks after withdrawal of doxycycline, sebaceous glands exhibit irregular maturation of sebocytes unlike in wild-type mice

enlarged sebaceous gland ( J:145312 )

• six weeks after withdrawal of doxycycline, sebaceous glands appear enlarged and displaced

sebaceous gland hyperplasia ( J:145312 )

• six weeks after withdrawal of doxycycline, sebaceous glands exhibit hyperplasia unlike in wild-type mice

sebaceous gland hypoplasia ( J:145312 )

• 17 weeks after doxycycline withdrawal, sebaceous glands appear hypoplastic

immune system

periodontium inflammation ( J:145312 )

• following doxycycline withdrawal at birth, mice exhibit spread of inflammation into the periodontal ligament and surrounding bony structure unlike in wild-type mice

• however, no changes were seen in the upper jaws

skin inflammation ( J:145312 )

• six weeks after withdrawal of doxycycline, inflammatory cells infiltrate the dermis unlike in wild-type mice

integument

abnormal sebocyte differentiation ( J:145312 )

• six weeks after withdrawal of doxycycline, sebaceous glands exhibit irregular maturation of sebocytes unlike in wild-type mice

enlarged sebaceous gland ( J:145312 )

• six weeks after withdrawal of doxycycline, sebaceous glands appear enlarged and displaced

sebaceous gland hyperplasia ( J:145312 )

• six weeks after withdrawal of doxycycline, sebaceous glands exhibit hyperplasia unlike in wild-type mice

sebaceous gland hypoplasia ( J:145312 )

• 17 weeks after doxycycline withdrawal, sebaceous glands appear hypoplastic

skin inflammation ( J:145312 )

• six weeks after withdrawal of doxycycline, inflammatory cells infiltrate the dermis unlike in wild-type mice

sparse hair ( J:145312 )

• following doxycycline withdrawal, mice have thin coats

• however, hair follicle number is normal

abnormal hypodermis morphology ( J:145312 )

• absent 17 weeks after withdrawal of doxycycline

abnormal hair follicle morphology ( J:145312 )

• six weeks after withdrawal of doxycycline

abnormal dermal layer morphology ( J:145312 )

• six weeks after withdrawal of doxycycline, inflammatory cells infiltrate the dermis unlike in wild-type mice

• 17 weeks after doxycycline withdrawal, the dermis exhibits fibrosis unlike in wild-type mice

hyperkeratosis ( J:145312 )

• six weeks after withdrawal of doxycycline

abnormal epidermis stratum granulosum morphology ( J:145312 )

• six weeks after withdrawal of doxycycline, mice exhibit hypergranulosis unlike wild-type mice

epidermal hyperplasia ( J:145312 )

• six weeks after withdrawal of doxycycline, mice exhibit slight to moderate hyperplasia of the interfollicular epidermis unlike in wild-type mice

• after withdrawal of doxycycline, some areas exhibit severe hyperplasia unlike in wild-type mice

skin lesions ( J:145312 )

• six weeks after withdrawal of doxycycline, mice exhibit skin lesions of varying severity unlike in wild-type mice

skin fibrosis ( J:145312 )

• 17 weeks after doxycycline withdrawal, the dermis exhibits fibrosis unlike in wild-type mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
progeria		DOID:3911	OMIM:176670	J:145312