mortality/aging
• 70% of mice die by E14.5
|
• 8% of mice die by E12.5
|
nervous system
N |
• unlike in mice homozygous for other Ift88 alleles, midbrain and telencephalon cilia are normal
|
• at E12.5, mice exhibit EGFP+ bulges in the pial surface of the forebrain unlike in wild-type mice
|
• at E12.5, mice exhibit disorganization of the dorsal telecephalon such that the hippocampal primordium and cortical hem can not be identified
• however, cell types are specified normally and cilia in the telencechaplon appear normal
• at E11.5, the telencephalon is shortened along its rostral-caudal axis compared to in wild-type mice
|
• at E11.5, the dorsal midline exhibits reduced invagination and disorganization, invaginations of the cortical ventricular zone and subpial heterotopias
• at E12.5, the ventricular zone is kinked and folded upon itself unlike in wild-type mice
• however, development of the medial and lateral ganglionic eminences and the number of mitotic cells in the ventricular zone cortex or located greater than 30 um basally from the ventricular zone cortex and ganglionic eminences are normal
• at E11.5 and E12.5, ganglionic eminences exhibit an increase in mitotic cells compared to in wild-type mice
• at the caudal level the separation between the diencephalons and dorsal telencephalon is lost
• numerous rosettes are detected in the dorsal telencephalic-diencephalic boundary region that contain mixture of ventricular, eminentia thalami and telencephalic cells
|
exencephaly
(
J:142500
)
• in 10% of mice at E12.5
|
cardiovascular system
• mice exhibit pulmonary artery atresia
|
• between E12.5 and E16.5 mice exhibit numerous cardiovascular development defects including arteria lusoria, interventricular and interatrial septal defects, dextrotransposition of
the common truncus arteriosus, and persistent truncus arteriosus
|
• instead of emptying into the right atrium, the coronary sinus emptied into the common atrium
|
• abnormal drainage of the right pulmonary vein into the inferior vena cava was observed
|
• common atrium
|
respiratory system
N |
• unlike in mice homozygous for other Ift88 alleles, bronchial cilia are normal
|
• between E12.5 and E16.5
|
• mice exhibit severe lung hypoplasia with minor arborization of the bronchial tree and left lung aplasia
|
vision/eye
limbs/digits/tail
polydactyly
(
J:142500
)
• in all mice in both fore- and hindlimbs
|