Severe facial clefting in Insig-deficient mouse embryos caused by sterol accumulation and reversed by lovastatin
J. Clin. Invest. Luke J. Engelking, et al. 116:2356 doi:10.1172/JCI28988 [
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Figure 3
Histology of control (
A,
D,
G, and
J) and
Insig-DKO embryos with cleft palate (
B,
E,
H, and
K) or cleft face (
C,
F,
I, and
L) at 18. dpc. (
A–
F) Palate phenotype. The secondary palate was intact in control (
A and
D) but absent in
Insig-DKO embryos (
B,
C,
E, and
F). The lateral margins of the palatal shelves are indicated by arrows. In
Insig-DKO embryos with cleft face (
C), the nasal septum was split, and the brain was displaced rostrally. (
G–
L) Middle and inner ear phenotype. Meckel cartilage (mc) and malleus (m) were normal in the cleft palate
Insig-DKO embryo (
H) but rudimentary in the cleft face embryo (
I). Stapes (s) and the stapedial artery (sa) were absent or vestigial in
Insig-DKO embryos (
K and
L). Compared with that of control embryos (
J), the pars canalicularis (pca) of the otic capsule in the
Insig-DKO embryos migrated rostral-ventrally (
K and
L). pa, palate; pco, pars cochlearis; tg, tongue. Scale bars: 0.5 mm.
