7-Dehydrocholesterol–dependent proteolysis of HMG-CoA reductase suppresses sterol biosynthesis in a mouse model of Smith-Lemli-Opitz/RSH syndrome
J. Clin. Invest. Barbara U. Fitzky, et al. 108:905 doi:10.1172/JCI12103 [
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Figure 5Histological sections of control mouse lung, (
a) ×10 and (
b) ×40, and
Dhcr7–/– mouse lung, (
c) ×10 and (
d) ×40, illustrating lung hypoplasia in the latter. Especially noteworthy is the excessive cellular density, the failure of the lungs to fill the pleural cavity (*), and their immature appearance.