mortality/aging
• about 90% survive to weaning
|
nervous system
• substantial but still incomplete rescue of brain defects
|
• although the cerebellum is grossly normal, cerebellar folding is somewhat delayed
• one mouse studied lacks the first anterior fold and has an aberrant 8th fold
• half of animals studied have the first two folds fused and a posterior shift of the eighth fold
|
• grossly normal but slight truncation is seen in some animals
|
limbs/digits/tail
• ventrally restricted eccrine glands are largely absent
|
• flexor digitorum and profundus tendons are present but appear to end blindly in the distal soft tissue of most digits
(J:34307)
• middle digits less severely affected
(J:50311)
• display the double dorsal morphology of En1tm1Alj
(J:50311)
• posterior paws less affected than anterior paws
(J:50311)
|
• hyperpigmentation developing at 3-4 weeks of age
|
polydactyly
(
J:50311
)
• postaxial
• ectopic ventral digits on both fore and hind paws
|
syndactyly
(
J:50311
)
• variable occurrence of fusion of digits is observed at 14 months
|
• at 3 months of age, nail/pad hybrid structures associated with metatarsal pads are clearly visible
|
• ventral skeletal elements such as falciform and sesamoid cartilages (bones) are absent or incompletely formed
|
skeleton
N |
• defects in skeletal structures of the body wall are rescued (sternum, ribs)
|
• ventral skeletal elements such as falciform and sesamoid cartilages (bones) are absent or incompletely formed
|
• variable occurrence of fusion of digits is observed at 14 months
|
pigmentation
• hyperpigmentation developing at 3-4 weeks of age
|
endocrine/exocrine glands
• ventrally restricted eccrine glands are largely absent
|
integument
• ventrally restricted eccrine glands are largely absent
|
• ventral nail-like structures developing at 3-4 weeks of age
|
• at 14 months, ectopic ventral hairs on distal limb
|
• hyperpigmentation developing at 3-4 weeks of age
|