TABLE 1.

DHCEO and sterol concentrations in Neuro2a cells, HFs, and brains from mice at embryonic day 20

	Neuro2a		Human fibroblast		E20 mouse brain
	Control	Dhcr7-def	Control	SLOS	WT	Het	KO
DHCEO (ng/mg)	0	3.51 ± 0.08a	39.5 ± 8.5	333 ± 115a	0.014 ± 0.004	0.039 ± 0.013b	1.45 ± 0.16a
7-DHC (μg/mg)	–	3.09 ± 0.02a	2.05 ± 0.43	19.1 ± 1.3a	–	–	3.21 ± 0.33a
Chol (μg/mg)	34.6 ± 5.5	34.4 ± 8.4	108 ± 21	65 ± 10c	3.12 ± 0.60	2.97 ± 0.12	0.42 ± 0.07a
7-DHC/Chol	–	0.093 ± 0.022a	0.019 ± 0.002	0.30 ± 0.04a	–	–	7.76 ± 0.72a

Per mg of protein for Neuro2a and HF; per mg of tissue for mouse brain (wet weight); statistical analyses are all relative to Control or WT; n = 3 for all Neuro2a and mouse samples; n = 6 for HF (triplicate of two different cell lines for control or SLOS, see Materials and Methods). –, Not available due to analytical limitation; WT, wild-type; Het, Dhcr7^+/−; KO, Dhcr7^−/−.

^ap < 0.0005.

^bp < 0.05.

^cp < 0.005.