Phenotypes associated with this allele

• Allele Symbol: Fli1^tm1Matr
• Allele Name: targeted mutation 1, Maria Trojanowska
• Allele ID: MGI:4441373
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Fli1^tm1Matr/Fli1^tm1Matr Tg(Tek-cre)12Flv/0	B6.Cg-Fli1^tm1Matr Tg(Tek-cre)12Flv	MGI:4441385
cn2	Fli1^tm1Matr/Fli1^tm1Matr Tg(Tek-cre)12Flv/Tg(Tek-cre)12Flv	B6.Cg-Fli1^tm1Matr Tg(Tek-cre)12Flv	MGI:4441395

Genotype
MGI:4441385

cn1

• Allelic Composition: Fli1^tm1Matr/Fli1^tm1Matr; Tg(Tek-cre)12Flv/0
• Genetic Background: B6.Cg-Fli1^tm1Matr Tg(Tek-cre)12Flv

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

cardiovascular system

abnormal blood vessel morphology ( J:158658 )

• dermal vasculature is disorganized with irregular vessel diameters unlike in wild-type mice

• dermal capillaries, arterioles, and venules exhibit increased vessel diameter compared to in wild-type mice

abnormal arteriole morphology ( J:158658 )

• dermal capillaries, arterioles, and venules exhibit increased vessel diameter compared to in wild-type mice

• arterioles exhibit a 65% increase in endothelial cells and pericytes compared to in wild-type mice

increased vascular endothelial cell number ( J:158658 )

• arterioles exhibit a 65% increase in endothelial cells and pericytes compared to in wild-type mice

• however, endothelial cell proliferation or apoptosis rates are normal

dilated capillary ( J:158658 )

• capillaries are dilated in the skin vascular network compared to in wild-type mice

• dermal capillaries, arterioles, and venules exhibit increased vessel diameter compared to in wild-type mice

abnormal pericyte morphology ( J:158658 )

• arterioles exhibit a 65% increase in endothelial cells and pericytes compared to in wild-type mice

• pericyte/vascular smooth muscle cell coverage is decreased compared to in wild-type mice

abnormal vascular smooth muscle morphology ( J:158658 )

• pericyte/vascular smooth muscle cell coverage is decreased compared to in wild-type mice

abnormal venule morphology ( J:158658 )

• dermal capillaries, arterioles, and venules exhibit increased vessel diameter compared to in wild-type mice

vascular stenosis ( J:158658 )

• in arterioles of dermal vascular networks

aneurysm ( J:158658 )

• microaneurysms are observed in the skin vascular network compared with wild-type mice

hemorrhage ( J:158658 )

• mice are more hemorrhagic against mechanical force such as cervical dislocation and surgical procedure compared with similarly treated wild-type mice

increased vascular permeability ( J:158658 )

• skin vasculature on the ears and back exhibits increased permeability compared with wild-type mice

• skin vascular leakage is more severe when ears are treated with mineral oil compared with wild-type ears treated with mustard oil

growth/size/body

decreased body weight ( J:158658 )

• from P3 until 3 months of age

muscle

abnormal vascular smooth muscle morphology ( J:158658 )

• pericyte/vascular smooth muscle cell coverage is decreased compared to in wild-type mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
systemic scleroderma		DOID:418	OMIM:181750	J:158658

Genotype
MGI:4441395

cn2

• Allelic Composition: Fli1^tm1Matr/Fli1^tm1Matr; Tg(Tek-cre)12Flv/Tg(Tek-cre)12Flv
• Genetic Background: B6.Cg-Fli1^tm1Matr Tg(Tek-cre)12Flv

mortality/aging

perinatal lethality, incomplete penetrance ( J:158658 )

prenatal lethality, complete penetrance ( J:158658 )

• fewer than expected mice are produced likely due to some embryonic and early perinatal loss