animal

Gene knockout

Age/weight

BAV

Significant hemodynamic stenosis?

Histopathological changes of AV

C57BL/6J background mice [95]

Chm-I

8 and 20 weeks of age

Not known

Not known

Calcification, Neoangiogenesis, Lipid deposition

in C57BL/6J but not 129S1/SvImJ mice [150]

EGFRWa2/Wa2

≤15 months

Not known

Yes, but background strain dependent

Cellular proliferation, ectopic cartilage for mation , extensive calcification, inflammatory infiltrate, Fibrosis

mixed 129/SvEv×BALB/cBy background mice [96]

Madh6-/-

4 - 6 months

Not known

Yes, may affect resistance vessels

Calcification, ossification, Excessive proliferation of mesenchymal cells

C57BL/6 background mice [72]

MGPm1/MGPm1 (-/-)

After birth

Not known

Not known

Calcification

C57BL/6J background mice [151]

eNOS-/-

25 to 30 g/embryos at day 13.5 of gestation

Bicuspid aortic valves in0~40% of mice

Not known

Bicuspid aortic valves in0~40% of mice

mice [152]

NOS3-/-; Notch1+/-

6 - 8 weeks

Aortic valve malformations occur in nearly 100% of cases, the most common being BAV

highly penetrate BAV and develop hemodynamically significant aortic valve stenosis and regurgitation

Calcification, aortic valve malformations

C57BL/6 background mice [153]

Notch1+/-

10 months of age

No

No

Calcification

N1+/ mTRWT mice (generation 1-3) [92]

Notch+/-mTRG2

≥1.5 months

Not known

No

Calcification

mice [91]

Notch1 (mutation)

After birth

Relevant, specific data cannot be speculated

Not known

Calcification, Bicuspid aortic valves

C57BL/6 background mice [90]

Postn-/-

6 or 10 months

Not known

Not known

Calcification, Fibrosis

C57BL/6 background mice [154]

Postn-/-

12 weeks

Not known

No

Reduced valve thickening, Calcification, fibrosis