mortality/aging
|
|
• after P17, mice are so severely malformed that they are sacrificed
|
limbs/digits/tail
|
|
• cilia on limb mesenchyme are shorted than in wild-type mice
• axoneme doublets are missing in the limb mesenchyme cilia unlike in wild-type mice
• however, length of cilia on the apical ectodermal ridge and ventral ectoderm is normal
|
polydactyly
(
J:168038
)
|
|
• severe with 7 to 9 unpatterned digits on each forelimb and a single extra digit on each hindlimb
|
syndactyly
(
J:168038
)
|
|
• in 2 of 8 mice
|
|
|
• at E16.5, the bone collar of the humerus is almost absent unlike in wild-type mice
|
short radius
(
J:168038
)
|
|
|
short ulna
(
J:168038
)
|
|
|
short femur
(
J:168038
)
|
|
|
short fibula
(
J:168038
)
|
|
|
short tibia
(
J:168038
)
|
|
|
embryo
|
|
• cilia on limb mesenchyme are shorted than in wild-type mice
• axoneme doublets are missing in the limb mesenchyme cilia unlike in wild-type mice
• however, length of cilia on the apical ectodermal ridge and ventral ectoderm is normal
|
skeleton
|
|
• at E16.5, the bone collar of the humerus is almost absent unlike in wild-type mice
|
|
|
• at P0, the central ossified diaphysis is reduced compared to in wild-type mice
|
|
|
• slightly disorganized
|
short radius
(
J:168038
)
|
|
|
short ulna
(
J:168038
)
|
|
|
short femur
(
J:168038
)
|
|
|
short fibula
(
J:168038
)
|
|
|
short tibia
(
J:168038
)
|
|
|
|
|
• reduced in the limbs at E16.5
|
|
|
• at E16.5, chondrocyte proliferation in the proximal ulna is decreased compared to in wild-type mice
• hypertrophic chondrocytes exhibit premature differentiation compared to in wild-type mice
• however, levels of chondrocyte apoptosis are normal
|


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