Table 1.
Plasma ketosterol concentrations
| 7α-hydroxy-4- cholesten-3-onea,b (ng/ml) |
7α12α-dihydroxy-4- cholesten-3-onea,b (ng/ml) |
7α12α-dihydroxy-5β- cholestan-3-onea (ng/ml) |
5α- cholestanolb,c (μg/ml) |
|
|---|---|---|---|---|
| CTX-case described plasma | 795 | 1548 | 1004 | 8.4 |
| Untreated CTX-affected adult plasma (n=10) | 1174 ± 711d [204-1828] | 1545 ± 1144d [57-2420] | 498 ± 310d [100-1004] | 31 ± 19 [8.4-66] |
| Unaffected adult plasma (n=20) | 6.4 ± 5.3e [1.6-22] | 0.4 ± 0.3e [0.1-0.8] | 10 ± 5.5e [0-29] | 1.3 [0.8-1.8]f |
The mean concentration ± S.D. and [range of results] are given
a
non-hydrolyzed free sterol.
b
this data has been reported previously [9].
c
hydrolyzed total sterol; quantification by GC-MS.
d
CTX plasma samples with concentrations >250 ng/ml were diluted and re-analyzed.
e
Calculated outside the quantifiable range.