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 4Palates were unremarkable in newborn
Dhcr7+/–and
Dhcr7+/+ mice (
a) but were frequently cleft in
Dhcr7–/– newborns (
b), while bladders were of normal size in controls (
c) but were nearly always distended (indicated by arrows in the opened abdomen) in
Dhcr7–/– mice (
d). ×10.
