Please wait a moment until all data is loaded. This message will disappear when all data is loaded.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
(24Z)-5alpha-stigmast-7,24(241)-dien-3beta-ol + O2 + reduced acceptor
(24Z)-stigmasta-5,7,24(241)-trien-3-beta-ol + acceptor + H2O
-
-
-
?
(3beta,24R)-ergost-7-en-3-ol + ferrocytochrome b5 + O2 + H+
(3beta,24R)-ergosta-5,7-dien-3-ol + ferricytochrome b5 + H2O
-
-
-
-
?
14-methylfecosterol + ferrocytochrome b5 + O2 + H+
14-methylergosta-5,8,24(28)-trien-3beta-ol + ferricytochrome b5 + H2O
-
-
-
?
5alpha-cholest-22-en-3beta-ol + ferrocytochrmoe b5 + H+ + O2
cholesta-5,22-dien-3beta-ol + ferricytochrome b5 + 2 H2O
5alpha-cholest-7-en-3beta-ol + 2 ferrocytochrome b5 + O2 + 2 H+
cholesta-5,7-dien-3beta-ol + 2 ferricytochrome b5 + 2 H2O
5alpha-cholest-7-en-3beta-ol + ferrocytochrmoe b5 + H+ + O2
cholesta-5,7-dien-3beta-ol + ferricytochrome b5 + H2O
NADH is more efficient than NADPH
-
-
?
5alpha-cholest-7-en-3beta-ol + ferrocytochrome b5 + H+ + O2
cholesta-5,7-dien-3beta-ol + ferricytochrome b5 + 2 H2O
5alpha-cholest-7-en-3beta-ol + ferrocytochrome b5 + O2 + H+
? + ferricytochrome b5 + H2O
-
-
-
-
?
5alpha-cholesta-7,22-dien-3beta-ol + ferrocytochrome b5 + H+ + O2
cholesta-5,7,22-trien-3beta-ol + ferricytochrome b5 + 2 H2O
5alpha-cholestan-3beta-ol + ferrocytochrome b5 + H+ + O2
cholest-5-en-3beta-ol + ferricytochrome b5 + 2 H2O
5alpha-ergost-7,24(241)-dien-3beta-ol + O2 + ferrocytochrome b5
ergosta-5,7,24(241)-trien-3beta-ol + H2O2 + ferricytochrome b5
5alpha-ergosta-7,22-dien-3beta-ol + ferrocytochrome b5 + O2 + H+
ergosterol + ferricytochrome b5 + H2O
5alpha-ergosta-7,24-dien-3beta-ol + ferrocytochrome b5 + O2 + H+
ergosta-5,7,24-trien-3beta-ol + ferricytochrome b5 + H2O
5alpha-ergosta-7-en-3beta-ol + ferrocytochrome b5 + O2 + H+
ergosta-5,7-dien-3beta-ol + ferricytochrome b5 + H2O
a DELTA7-sterol + 2 ferrocytochrome b5 + O2 + 2 H+
a DELTA5,7-sterol + 2 ferricytochrome b5 + 2 H2O
-
-
-
?
avenasterol + ferrocytochrome b5 + O2 + H+
?
-
-
-
-
?
cholest-7-en-3beta-ol + ferrocytochrome b5 + O2 + H+
cholest-5,7-dien-3beta-ol + ferricytochrome b5 + H2O
-
-
-
-
?
cholesta-7,24-dien-3beta-ol + 2 ferrocytochrome b5 + O2 + 2 H+
7-dehydrodesmosterol + 2 ferricytochrome b5 + 2 H2O
DELTA7-avenasterol + O2 + ferrocytochrome b5
DELTA5,7-avenasterol + H2O + ferricytochrome b5
-
-
-
-
?
DELTA7-campesterol + O2 + ferrocytochrome b5
DELTA5,7-campesterol + H2O + ferricytochrome b5
-
-
-
-
?
DELTA7-sitosterol + O2 + ferrocytochrome b5
DELTA5,7-sitosterol + H2O + ferricytochrome b5
-
-
-
-
?
DELTA7-sterol + ferrocytochrome b5 + O2 + H+
DELTA5,7-sterol + ferricytochrome b5 + H2O
-
-
-
-
?
DELTA7-ststigmast-7-en-3beta-ol + ferrocytochrome b5 + O2 + H+
stigmast-5,7-dien-3beta-ol + ferricytochrome b5 + H2O
-
-
-
-
?
episterol + ferrocytochrome b5 + O2 + H+
? + ferricytochrome b5 + H2O
-
-
-
-
?
episterol + ferrocytochrome b5 + O2 + H+
ergosta-5,7,24(28)-trien-3beta-ol + ferricytochrome b5 + 2 H2O
episterol + O2 + ferrocytochrome b5
DELTA5,7-episterol + H2O + ferricytochrome b5
-
-
-
-
?
ergosta-7,22-dien-3beta-ol + ferrocytochrome b5 + O2 + H+
ergosta-5,7,22-trien-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
ergosta-7,22-dien-3beta-ol + ferrocytochrome b5 + O2 + H+
ergosterol + ferricytochrome b5 + 2 H2O
-
-
-
?
ergosta-7,24(28)-dien-3beta-ol + ferrocytochrome b5 + O2 + H+
ergosta-5,7,24(28)-trien-3beta-ol + ferricyctochrome b5 + H2O
-
-
-
-
?
ergosta-7-en-3beta-ol + ferrocytochrome b5 + O2 + H+
ergosta-5,7-dien-3beta-ol + ferricyctochrome b5 + H2O
-
-
-
-
?
ergosta-8,22,24(28)-trien-3beta-ol + ferrocytochrome b5 + O2 + H+
ergosta-5,8,22,24(28)-tetraen-3beta-ol + ferricyctochrome b5 + H2O
-
-
-
-
?
ergosta-8,22-dien-3beta-ol + ferrocytochrome b5 + O2 + H+
ergosta-5,8,22-trien-3beta-ol + ferricyctochrome b5 + H2O
-
-
-
-
?
ergosterol + ferrocytochrome b5 + O2 + H+
lanosterol + ferricyctochrome b5 + H2O
-
-
-
-
?
fecosterol + ferrocytochrome b5 + O2 + H+
ergosta-5,7,24(28)-trien-3beta-ol + ferricyctochrome b5 + H2O
-
-
-
-
?
lanosterol + ferrocytochrome b5 + O2 + H+
? + ferricytochrome b5 + H2O
lanosterol + ferrocytochrome b5 + O2 + H+
lanosta-5,8,24-trien-3beta-ol + ferricyctochrome b5 + H2O
-
-
-
-
?
lathosterol + 2 ferrocytochrome b5 + O2 + 2 H+
7-dehydrocholesterol + 2 ferricytochrome b5 + 2 H2O
lathosterol + ferrocytochrome b5 + O2 + H+
7-dehydrocholesterol + ferricytochrome b5 + H2O
-
-
-
-
?
lathosterol + ferrocytochrome b5 + O2 + H+
?
-
-
-
-
?
lathosterol + O2 + ferrocytochrome b5
7-dehydrocholesterol + H2O + ferricytochrome b5
-
-
-
-
?
stigmast-7-en-3beta-ol + ferrocytochrome b5 + O2 + H+
stigmast-5,7-dien-3beta-ol + ferricyctochrome b5 + H2O
-
-
-
-
?
stigmasta-7,22-dien-3beta-ol + ferrocytochrome b5 + O2 + H+
stigmast-5,7,22-trien-3beta-ol + ferricyctochrome b5 + H2O
-
-
-
-
?
zymosterol + ferrocytochrome b5 + O2 + H+
cholesta-5,8,24-trien-3beta-ol + ferricyctochrome b5 + H2O
-
-
-
-
?
additional information
?
-
5alpha-cholest-22-en-3beta-ol + ferrocytochrmoe b5 + H+ + O2
cholesta-5,22-dien-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-cholest-22-en-3beta-ol + ferrocytochrmoe b5 + H+ + O2
cholesta-5,22-dien-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-cholest-7-en-3beta-ol + 2 ferrocytochrome b5 + O2 + 2 H+
cholesta-5,7-dien-3beta-ol + 2 ferricytochrome b5 + 2 H2O
-
i.e. lathosterol
-
?
5alpha-cholest-7-en-3beta-ol + 2 ferrocytochrome b5 + O2 + 2 H+
cholesta-5,7-dien-3beta-ol + 2 ferricytochrome b5 + 2 H2O
-
i.e. lathosterol
-
?
5alpha-cholest-7-en-3beta-ol + 2 ferrocytochrome b5 + O2 + 2 H+
cholesta-5,7-dien-3beta-ol + 2 ferricytochrome b5 + 2 H2O
-
i.e. lathosterol
-
?
5alpha-cholest-7-en-3beta-ol + 2 ferrocytochrome b5 + O2 + 2 H+
cholesta-5,7-dien-3beta-ol + 2 ferricytochrome b5 + 2 H2O
-
enzyme is rather a mixed function oxidase which requires O2 and NAD(P)H for activity, than a dehydrogenase
-
?
5alpha-cholest-7-en-3beta-ol + 2 ferrocytochrome b5 + O2 + 2 H+
cholesta-5,7-dien-3beta-ol + 2 ferricytochrome b5 + 2 H2O
-
NADPH, 40% of activity with NADH
-
?
5alpha-cholest-7-en-3beta-ol + 2 ferrocytochrome b5 + O2 + 2 H+
cholesta-5,7-dien-3beta-ol + 2 ferricytochrome b5 + 2 H2O
-
cholesterol biosynthesis
-
?
5alpha-cholest-7-en-3beta-ol + 2 ferrocytochrome b5 + O2 + 2 H+
cholesta-5,7-dien-3beta-ol + 2 ferricytochrome b5 + 2 H2O
-
cholesterol biosynthesis
-
?
5alpha-cholest-7-en-3beta-ol + 2 ferrocytochrome b5 + O2 + 2 H+
cholesta-5,7-dien-3beta-ol + 2 ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-cholest-7-en-3beta-ol + ferrocytochrome b5 + H+ + O2
cholesta-5,7-dien-3beta-ol + ferricytochrome b5 + 2 H2O
NADH is more efficient than NADPH
-
-
?
5alpha-cholest-7-en-3beta-ol + ferrocytochrome b5 + H+ + O2
cholesta-5,7-dien-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-cholest-7-en-3beta-ol + ferrocytochrome b5 + H+ + O2
cholesta-5,7-dien-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-cholesta-7,22-dien-3beta-ol + ferrocytochrome b5 + H+ + O2
cholesta-5,7,22-trien-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-cholesta-7,22-dien-3beta-ol + ferrocytochrome b5 + H+ + O2
cholesta-5,7,22-trien-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-cholestan-3beta-ol + ferrocytochrome b5 + H+ + O2
cholest-5-en-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-cholestan-3beta-ol + ferrocytochrome b5 + H+ + O2
cholest-5-en-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-ergost-7,24(241)-dien-3beta-ol + O2 + ferrocytochrome b5
ergosta-5,7,24(241)-trien-3beta-ol + H2O2 + ferricytochrome b5
-
-
-
?
5alpha-ergost-7,24(241)-dien-3beta-ol + O2 + ferrocytochrome b5
ergosta-5,7,24(241)-trien-3beta-ol + H2O2 + ferricytochrome b5
-
-
-
?
5alpha-ergosta-7,22-dien-3beta-ol + ferrocytochrome b5 + O2 + H+
ergosterol + ferricytochrome b5 + H2O
-
-
-
?
5alpha-ergosta-7,22-dien-3beta-ol + ferrocytochrome b5 + O2 + H+
ergosterol + ferricytochrome b5 + H2O
-
-
-
?
5alpha-ergosta-7,24-dien-3beta-ol + ferrocytochrome b5 + O2 + H+
ergosta-5,7,24-trien-3beta-ol + ferricytochrome b5 + H2O
-
-
-
?
5alpha-ergosta-7,24-dien-3beta-ol + ferrocytochrome b5 + O2 + H+
ergosta-5,7,24-trien-3beta-ol + ferricytochrome b5 + H2O
-
-
-
?
5alpha-ergosta-7-en-3beta-ol + ferrocytochrome b5 + O2 + H+
ergosta-5,7-dien-3beta-ol + ferricytochrome b5 + H2O
-
-
-
?
5alpha-ergosta-7-en-3beta-ol + ferrocytochrome b5 + O2 + H+
ergosta-5,7-dien-3beta-ol + ferricytochrome b5 + H2O
-
-
-
?
cholesta-7,24-dien-3beta-ol + 2 ferrocytochrome b5 + O2 + 2 H+
7-dehydrodesmosterol + 2 ferricytochrome b5 + 2 H2O
-
-
-
-
?
cholesta-7,24-dien-3beta-ol + 2 ferrocytochrome b5 + O2 + 2 H+
7-dehydrodesmosterol + 2 ferricytochrome b5 + 2 H2O
-
-
-
-
?
episterol + ferrocytochrome b5 + O2 + H+
ergosta-5,7,24(28)-trien-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
-
?
episterol + ferrocytochrome b5 + O2 + H+
ergosta-5,7,24(28)-trien-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
-
?
lanosterol + ferrocytochrome b5 + O2 + H+
? + ferricytochrome b5 + H2O
-
-
-
?
lanosterol + ferrocytochrome b5 + O2 + H+
? + ferricytochrome b5 + H2O
-
-
-
?
lathosterol + 2 ferrocytochrome b5 + O2 + 2 H+
7-dehydrocholesterol + 2 ferricytochrome b5 + 2 H2O
-
-
-
-
?
lathosterol + 2 ferrocytochrome b5 + O2 + 2 H+
7-dehydrocholesterol + 2 ferricytochrome b5 + 2 H2O
-
-
-
-
?
additional information
?
-
-
enzyme deficiency leads to lathosterolosis, a defect of cholesterol biosynthesis, phenotype, detailed overview
-
-
?
additional information
?
-
-
the enzyme is involved in the post-squalene part of cholesterol biosynthesis
-
-
?
additional information
?
-
-
no activity with 4alpha-methyl-5alpha-cholest-7-en-3beta-ol, 4alpha,14alpha-dimethyl-5alpha-cholest-7-en-3beta-ol, cholestan-3beta-ol and cholest-8-en-3beta-ol
-
-
?
additional information
?
-
-
the specificity of maize C5(6)-desaturase favors DELTA7-sterols possessing a C24-methylene or ethylidene substituent compared to 24-ethyl-substituted DELTA7-sterols
-
-
?
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
(3beta,24R)-ergost-7-en-3-ol + ferrocytochrome b5 + O2 + H+
(3beta,24R)-ergosta-5,7-dien-3-ol + ferricytochrome b5 + H2O
-
-
-
-
?
5alpha-cholest-22-en-3beta-ol + ferrocytochrmoe b5 + H+ + O2
cholesta-5,22-dien-3beta-ol + ferricytochrome b5 + 2 H2O
5alpha-cholest-7-en-3beta-ol + 2 ferrocytochrome b5 + O2 + 2 H+
cholesta-5,7-dien-3beta-ol + 2 ferricytochrome b5 + 2 H2O
5alpha-cholest-7-en-3beta-ol + ferrocytochrome b5 + H+ + O2
cholesta-5,7-dien-3beta-ol + ferricytochrome b5 + 2 H2O
5alpha-cholesta-7,22-dien-3beta-ol + ferrocytochrome b5 + H+ + O2
cholesta-5,7,22-trien-3beta-ol + ferricytochrome b5 + 2 H2O
5alpha-cholestan-3beta-ol + ferrocytochrome b5 + H+ + O2
cholest-5-en-3beta-ol + ferricytochrome b5 + 2 H2O
a DELTA7-sterol + 2 ferrocytochrome b5 + O2 + 2 H+
a DELTA5,7-sterol + 2 ferricytochrome b5 + 2 H2O
-
-
-
?
cholest-7-en-3beta-ol + ferrocytochrome b5 + O2 + H+
cholest-5,7-dien-3beta-ol + ferricytochrome b5 + H2O
-
-
-
-
?
DELTA7-sterol + ferrocytochrome b5 + O2 + H+
DELTA5,7-sterol + ferricytochrome b5 + H2O
-
-
-
-
?
DELTA7-ststigmast-7-en-3beta-ol + ferrocytochrome b5 + O2 + H+
stigmast-5,7-dien-3beta-ol + ferricytochrome b5 + H2O
-
-
-
-
?
episterol + ferrocytochrome b5 + O2 + H+
? + ferricytochrome b5 + H2O
-
-
-
-
?
lathosterol + ferrocytochrome b5 + O2 + H+
7-dehydrocholesterol + ferricytochrome b5 + H2O
-
-
-
-
?
lathosterol + O2 + ferrocytochrome b5
7-dehydrocholesterol + H2O + ferricytochrome b5
-
-
-
-
?
additional information
?
-
5alpha-cholest-22-en-3beta-ol + ferrocytochrmoe b5 + H+ + O2
cholesta-5,22-dien-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-cholest-22-en-3beta-ol + ferrocytochrmoe b5 + H+ + O2
cholesta-5,22-dien-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-cholest-7-en-3beta-ol + 2 ferrocytochrome b5 + O2 + 2 H+
cholesta-5,7-dien-3beta-ol + 2 ferricytochrome b5 + 2 H2O
-
cholesterol biosynthesis
-
?
5alpha-cholest-7-en-3beta-ol + 2 ferrocytochrome b5 + O2 + 2 H+
cholesta-5,7-dien-3beta-ol + 2 ferricytochrome b5 + 2 H2O
-
cholesterol biosynthesis
-
?
5alpha-cholest-7-en-3beta-ol + ferrocytochrome b5 + H+ + O2
cholesta-5,7-dien-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-cholest-7-en-3beta-ol + ferrocytochrome b5 + H+ + O2
cholesta-5,7-dien-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-cholesta-7,22-dien-3beta-ol + ferrocytochrome b5 + H+ + O2
cholesta-5,7,22-trien-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-cholesta-7,22-dien-3beta-ol + ferrocytochrome b5 + H+ + O2
cholesta-5,7,22-trien-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-cholestan-3beta-ol + ferrocytochrome b5 + H+ + O2
cholest-5-en-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
5alpha-cholestan-3beta-ol + ferrocytochrome b5 + H+ + O2
cholest-5-en-3beta-ol + ferricytochrome b5 + 2 H2O
-
-
-
?
additional information
?
-
-
enzyme deficiency leads to lathosterolosis, a defect of cholesterol biosynthesis, phenotype, detailed overview
-
-
?
additional information
?
-
-
the enzyme is involved in the post-squalene part of cholesterol biosynthesis
-
-
?
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
(3S,20S)-20-N-(2-methylpropyl)carbamoylpregn-7-en-3beta-ol
-
specific
(3S,20S)-20-N-(2-methylpropyl)carbamoylpregn-7-en-3beta-yl acetate
-
specific for the enzyme. Prodrug, transformed into the 3beta-hydroxy derivative under in vitro conditions
(3S,20S)-20-N-butylcarbamoylpregn-7-en-3beta-ol
-
specific
(3S,20S)-20-N-butylcarbamoylpregn-7-en-3beta-yl acetate
-
specific for the enzyme. Prodrug, transformed into the 3beta-hydroxy derivative under in vitro conditions
(3S,20S)-20-N-ethylcarbamoylpregn-7-en-3beta-ol
-
-
(3S,20S)-20-N-ethylcarbamoylpregn-7-en-3beta-yl acetate
-
-
(3S,20S)-20-N-pentylcarbamoylpregn-7-en-3beta-ol
-
specific
(3S,20S)-20-N-pentylcarbamoylpregn-7-en-3beta-yl acetate
-
specific for the enzyme. Prodrug, transformed into the 3beta-hydroxy derivative under in vitro conditions
(3S,20S)-20-N-propylcarbamoylpregn-7-en-3beta-ol
-
-
(3S,20S)-20-N-propylcarbamoylpregn-7-en-3beta-yl acetate
-
-
(3S,20S)-20-N-tert-butylcarbamoylpregn-7-en-3beta-ol
-
specific
(3S,20S)-20-N-tert-butylcarbamoylpregn-7-en-3beta-yl acetate
-
specific for the enzyme. Prodrug, transformed into the 3beta-hydroxy derivative under in vitro conditions
2-(4-phenethylpiperazin-1-yl)-1-(pyridine-3-yl)ethanol
-
average content of 7-dehydrocholesterol in human primary hepatocytes after 2-(4-phenethylpiperazin-1-yl)-1-(pyridine-3-yl)ethanol treatment is, 34.16 times higher than that in nontreated cells, indicating that DHCR7 is the major target (minor targets are human sterol DELTA14-reductase, sterol DELTA24-reductase, and sterol C5-desaturase). In vitro kinetic measurements show that the half-life of 2-(4-phenethylpiperazin-1-yl)-1-(pyridine-3-yl)ethanol is at least 4.3 h. 2-(4-phenethylpiperazin-1-yl)-1-(pyridine-3-yl)ethanol does not induce CYP3A4 mRNA nor enzyme activity
4,5-dihydroxy-1,3-benzene-disulfonic acid
-
trivial name tiron, 0.5 mM, 80% inhibition
alpha-(2,4-dichlorophenyl)-alpha-phenyl-5-pyrimidine methanol
-
trivial name triarimol, weak inhibition
anti-cytochrome b5 antibodies
-
0.5 mg/mg protein. 36% inhibition
-
bathophenanthroline
-
0.5 mM, 71% inhibition
CYC (cyctochrome c)
-
-
-
dithiothreitol
-
1.0 mM, 55% inhibition
lathosterol side chain amides
-
synthesis and compound screening, influence of different sterol amides on inhibition of the enzyme, overview, lathosterol-derived amides with larger substituents, e.g. butyl, isobutyl, tert-butyl, pentyl, at the amide nitrogen are selective inhibitors of lathosterol oxidase, overview
-
menadione
-
32% residual actitivty at 0.05 mM
Salicylhydroxamic acid
-
-
salicylic hydroxamic acid
-
-
Tiron
-
21% residual activity at 1 mM
Triarimol
-
weak inhibition
1,10-phenanthroline
-
0.5 mM, 74% inhibition
CN-
0.003 mM, 50% inhibition of recombinant enzyme
CN-
-
0.5 mM, 95% inhibition
CN-
-
0.003-0015 mM, 50% inhibition
cyanide
-
cyanide
-
complete inhibition at 5 mM
additional information
-
cytotoxicity and inhibitor potencies for inhibition of total cholesterol biosynthesis, overview
-
additional information
-
the enzyme is not affected by N-ethylmaleimide and dithiothreitol
-
additional information
-
not inhibited by CO
-
additional information
-
not inhibited by CO
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
malfunction
-
pathophysiological mechanisms underlying lathosterolosis neurological symptoms are studies using quantitative proteomics analysis of lathosterolosis mouse brain tissue of Dhcr7 mutant mice. Multiple biological pathways are affected including alterations in mevalonate metabolism, apoptosis, glycolysis, oxidative stress, protein biosynthesis, intracellular trafficking, and cytoskeleton. Increased expression of isoprenoid and cholesterol synthetic enzymes is shown, suggesting that although cholesterol synthesis is impaired in both Dhcr7 mutant mice the synthesis of nonsterol isoprenoids may be increased and thus contribute to lathosterolosis pathology
malfunction
inactivation of sterol DELTA5,6-desaturase attenuates virulence in Candida albicans
malfunction
a mechanism of antifungal resistance in this organism affecting susceptibility to azole and echinocandin antifungals is found in a clinical isolate obtained from a patient with prosthetic valve endocarditis. Transcriptome analysis indicates differential expression of several genes in the resistant isolate, including upregulation of ergosterol biosynthesis pathway genes ERG2, ERG5, ERG6, ERG11, ERG24, ERG25, and UPC2. Whole-genome sequencing reveals that the resistant isolate possesses an ERG3 mutation resulting in a G111R amino acid substitution. Sterol profiles indicate a reduction in sterol desaturase activity as a result of this mutation. Replacement of both mutant alleles in the resistant isolate with the susceptible isolate's allele restores wild-type susceptibility to all azoles and echinocandins tested
malfunction
-
mice deleted for keratinocyte Sc5d lose the ability to increase circulating D3 following UVR exposure of the skin. Unlike in control mice, acute UVR exposure does not affect circulating D3 level in inducible Sc5dk14KOi mice. Phenotypes, overview
malfunction
-
a mechanism of antifungal resistance in this organism affecting susceptibility to azole and echinocandin antifungals is found in a clinical isolate obtained from a patient with prosthetic valve endocarditis. Transcriptome analysis indicates differential expression of several genes in the resistant isolate, including upregulation of ergosterol biosynthesis pathway genes ERG2, ERG5, ERG6, ERG11, ERG24, ERG25, and UPC2. Whole-genome sequencing reveals that the resistant isolate possesses an ERG3 mutation resulting in a G111R amino acid substitution. Sterol profiles indicate a reduction in sterol desaturase activity as a result of this mutation. Replacement of both mutant alleles in the resistant isolate with the susceptible isolate's allele restores wild-type susceptibility to all azoles and echinocandins tested
-
malfunction
-
mice deleted for keratinocyte Sc5d lose the ability to increase circulating D3 following UVR exposure of the skin. Unlike in control mice, acute UVR exposure does not affect circulating D3 level in inducible Sc5dk14KOi mice. Phenotypes, overview
-
metabolism
the enzyme is involved in ergosterol synthesis
metabolism
-
the enzyme is involved in the post squalene cholesterol and vitamin D3 synthesis, pathway overview
metabolism
-
the enzyme is involved in the post squalene cholesterol and vitamin D3 synthesis, pathway overview
-
physiological function
-
the enzyme is not essential for cell viability or the sparking function
physiological function
one gene essential to ergosterol biosynthesis is ERG3, which encodes the DELTA7-sterol-C5 (6)-desaturase responsible for introducing a double bond at C-5 in the B ring of episterol. DELTA7-sterol-C5(6)-desaturase is a membrane-bound enzyme that catalyzes introduction of a C-5 double bond into the B ring of DELTA7-sterols to yield the corresponding DELTA5,7-sterols
additional information
sterol composition of wild-type isolate and ERG3 mutants, overview
additional information
-
sterol composition of wild-type isolate and ERG3 mutants, overview
additional information
sterol composition of wild-type isolate and ERG3 mutants, overview
additional information
-
sterol composition of wild-type isolate and ERG3 mutants, overview
additional information
-
sterol composition of wild-type isolate and ERG3 mutants, overview
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
T114I |
Km-value for 5alpha-cholest-7-en-3beta-ol is 7.5fold higher than that of wild-type activity
L193R
the mutation impairs enzyme function
G234A
no activity
G234A
inactive mutant enzyme
G234D
2% of wild-type activity
H147E
no activity
H147E
inactive mutant enzyme
H147L
no activity
H147L
inactive mutant enzyme
H151E
no activity
H151E
inactive mutant enzyme
H151l
no activity
H151l
inactive mutant enzyme
H161E
no activity
H161E
inactive mutant enzyme
H161L
no activity
H161L
inactive mutant enzyme
H164L
no activity
H164L
inactive mutant enzyme
H165L
no activity
H165L
inactive mutant enzyme
H203L
reduced activity
H203L
13% of wild-type activity
H222E
reduced activity
H222E
6% of wild-type activity
H222L
no activity
H222L
inactive mutant enzyme
H238E
no activity
H238E
inactive mutant enzyme
H238L
no activity
H238L
inactive mutant enzyme
H241L
no activity
H241L
inactive mutant enzyme
H242L
no activity
H242L
inactive mutant enzyme
K115L
2times higher activity than wild-type
K115L
Km-value for 5alpha-cholest-7-en-3beta-ol is 2.8fold higher than that of wild-type activity
P175A
wild-type activity
P175A
as active as wild-type enzyme
P175V
4times higher activity than wild-type
P175V
Km-value for 5alpha-cholest-7-en-3beta-ol is 2.7fold higher than that of wild-type activity
P201A
25% of wild-type activity
P201A
20% of wild-type activity
T114S
28times higher activity than wild-type
T114S
Km-value for 5alpha-cholest-7-en-3beta-ol is 7.6fold higher than that of wild-type activity
G111R
naturally occurring mutation, the mutant is obtained from a patient with prosthetic valve endocarditis, the mutant shows reduction in sterol desaturase activity and resistance to azole and echinocandin antifungals, replacement of both mutant alleles in the resistant isolate with the susceptible isolate's allele restores wild-type susceptibility to all azoles and echinocandins tested.
G111R
-
naturally occurring mutation, the mutant is obtained from a patient with prosthetic valve endocarditis, the mutant shows reduction in sterol desaturase activity and resistance to azole and echinocandin antifungals, replacement of both mutant alleles in the resistant isolate with the susceptible isolate's allele restores wild-type susceptibility to all azoles and echinocandins tested.
-
additional information
mutant CAAL76 with a 13 bp deletion leading to a truncated enzyme (DELTA366-378) shows impaired enzyme function
additional information
an ERG3 deletion construct for Candida albicans is generated by amplifying an ApaI-XhoI containing fragment consisting of flanking regions upstream from positions -350 to +39 relative to the start codon of Candida albicans ERG3 using primers CaERG3A-F and CaERG3B-R, as well as a NotI-SacIIcontaining fragment consisting of flanking regions from positions +997 to +1677 downstream of the start codon using primers CaERG3C-F and CaERG3D-R. These upstream and downstream fragments are cloned upstream and downstream, respectively, of the SAT1-flipper cassette in plasmid pSFS2, resulting in pCaERG3M1
additional information
-
an ERG3 deletion construct for Candida albicans is generated by amplifying an ApaI-XhoI containing fragment consisting of flanking regions upstream from positions -350 to +39 relative to the start codon of Candida albicans ERG3 using primers CaERG3A-F and CaERG3B-R, as well as a NotI-SacIIcontaining fragment consisting of flanking regions from positions +997 to +1677 downstream of the start codon using primers CaERG3C-F and CaERG3D-R. These upstream and downstream fragments are cloned upstream and downstream, respectively, of the SAT1-flipper cassette in plasmid pSFS2, resulting in pCaERG3M1
additional information
an ERG3 deletion construct for Candida parapsilosis is generated by amplifying an ApaI-XhoI-containing fragment consisting of flanking regions upstream from positions -280 to +51 relative to the start codon of Candida parapsilosis ERG3 using primers ERG3-A and ERG3-B, as well as a NotI-SacII-containing fragment consisting of downstream flanking regions from positions +1047 to +1868 using primers ERG3-C and ERG3-D. These upstream and downstream fragments of ERG3 are cloned upstream and downstream, respectively, of the SAT1-flipper cassette in plasmid pSFS2 to result in plasmid p77ERG3
additional information
-
an ERG3 deletion construct for Candida parapsilosis is generated by amplifying an ApaI-XhoI-containing fragment consisting of flanking regions upstream from positions -280 to +51 relative to the start codon of Candida parapsilosis ERG3 using primers ERG3-A and ERG3-B, as well as a NotI-SacII-containing fragment consisting of downstream flanking regions from positions +1047 to +1868 using primers ERG3-C and ERG3-D. These upstream and downstream fragments of ERG3 are cloned upstream and downstream, respectively, of the SAT1-flipper cassette in plasmid pSFS2 to result in plasmid p77ERG3
additional information
-
an ERG3 deletion construct for Candida parapsilosis is generated by amplifying an ApaI-XhoI-containing fragment consisting of flanking regions upstream from positions -280 to +51 relative to the start codon of Candida parapsilosis ERG3 using primers ERG3-A and ERG3-B, as well as a NotI-SacII-containing fragment consisting of downstream flanking regions from positions +1047 to +1868 using primers ERG3-C and ERG3-D. These upstream and downstream fragments of ERG3 are cloned upstream and downstream, respectively, of the SAT1-flipper cassette in plasmid pSFS2 to result in plasmid p77ERG3
-
additional information
-
enzyme deficiency leads to lathosterolosis, a defect of cholesterol biosynthesis, phenotype, detailed overview
additional information
-
generation of mice carrying a modified allele of sterol C5-desaturase (Sc5d), the gene encoding the enzyme that converts lathosterol to 7-dehydrocholesterol (7-DHC) by blastocyst injection, causing a reading frame shift and thereby likely triggering nonsense mediated decay of the aberrant transcript, such that Sc5d expression can be inactivated using the Cre/lox site-specific recombination system. By crossing to mice with tissue-specific expression of Cre or CreER2 (Cre/estrogen receptor), two lines of transgenic mice are generated. One line has constitutive keratinocyte-specific inactivation of Sc5d (Sc5dk14KO). The other line (Sc5dk14KOi) has tamoxifen-inducible keratinocyte-specific inactivation of Sc5d. Breeding scheme, overview. Confirmation of SC5D loss in keratinocytes by IHC and by measuring vitamin D3 level in the blood
additional information
-
generation of mice carrying a modified allele of sterol C5-desaturase (Sc5d), the gene encoding the enzyme that converts lathosterol to 7-dehydrocholesterol (7-DHC) by blastocyst injection, causing a reading frame shift and thereby likely triggering nonsense mediated decay of the aberrant transcript, such that Sc5d expression can be inactivated using the Cre/lox site-specific recombination system. By crossing to mice with tissue-specific expression of Cre or CreER2 (Cre/estrogen receptor), two lines of transgenic mice are generated. One line has constitutive keratinocyte-specific inactivation of Sc5d (Sc5dk14KO). The other line (Sc5dk14KOi) has tamoxifen-inducible keratinocyte-specific inactivation of Sc5d. Breeding scheme, overview. Confirmation of SC5D loss in keratinocytes by IHC and by measuring vitamin D3 level in the blood
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Rahier, A.; Smith, M.; Taton, M.
The role of cytochrome b5 in 4alpha-methyl-oxidation and C5(6) desaturation of plant sterol precursors
Biochem. Biophys. Res. Commun.
236
434-437
1997
Zea mays
brenda
Kawata, S.; Traskos, J.M.; Gaylor, J.L.
Microsomal enzymes of cholesterol biosynthesis from lanosterol. Purification and characterization of DELTA 7-sterol 5-desaturase of rat liver microsomes
J. Biol. Chem.
260
6609-6617
1985
Rattus norvegicus
brenda
Grinstead, G.F.; Gaylor, J.L.
Total enzymic synthesis of cholesterol from 4,4,14alpha-trimethyl-5alpha-cholesta-8,24-dien-3beta-ol. Solubilization, resolution, and reconstitution of DELTA 7-sterol 5-desaturase
J. Biol. Chem.
257
13937-13944
1982
Rattus norvegicus
brenda
Ishibashi, T.; Bloch, K.
Intermembrane transfer of 5alpha-cholest-7-en-3beta-ol. Facilitation by supernatant protein (SCP)
J. Biol. Chem.
256
12962-12967
1981
Rattus norvegicus
brenda
Dempsey, M.E.; McCoy, K.E.; Barker, H.N.
Large scale purification and structural characterization of squalene and sterol carrier protein
J. Biol. Chem.
256
1867-1873
1981
Rattus norvegicus
brenda
Mitropoulos, K.A.; Gibbons, G.F.
Effect of triarimol on cholesterol biosynthesis in rat-liver subcellular
Biochem. Biophys. Res. Commun.
71
892-900
1976
Rattus norvegicus
brenda
Husselstein, T.; Schaller, H.; Gachotte, D.; Benveniste, P.
DELTA7-sterol-C5-desaturase: molecular characterization and functional expression of wild-type and mutant alleles
Plant Mol. Biol.
39
891-906
1999
Arabidopsis thaliana, Saccharomyces cerevisiae, Nicotiana tabacum, Zea mays
brenda
Nishi, S.; Nishino, H.; Ishibashi, T.
cDNA cloning of the mammalian sterol C5-desaturase and the expression in yeast mutant
Biochim. Biophys. Acta
1490
106-108
2000
Homo sapiens, Mus musculus
brenda
Taton, M.; Husselstein, T.; Benveniste, P.; Rahier, A.
Role of highly conserved residues in the reaction catalyzed by recombinant DELTA7-sterol-C5(6)-desaturase studied by site-directed mutagenesis
Biochemistry
39
701-711
2000
Arabidopsis thaliana (Q39208), Arabidopsis thaliana
brenda
Rahier, A.; Benveniste, P.; Husselstein, T.; Taton, M.
Biochemistry and site-directed mutational analysis of DELTA7-sterol-C5(6)-desaturase
Biochem. Soc. Trans.
28
799-803
2000
Zea mays
brenda
Sugawara, T.; Fujimoto, Y.; Ishibashi, T.
Molecular cloning and structural analysis of human sterol C5 desaturase
Biochim. Biophys. Acta
1533
277-284
2001
Homo sapiens
brenda
Rahier, A.
Deuterated DELTA7-cholestenol analogues as mechanistic probes for wild-type and mutated DELTA7-sterol-C5(6)-desaturase
Biochemistry
40
256-267
2001
Arabidopsis thaliana, Zea mays
brenda
Krakowiak, P.A.; Wassif, C.A.; Kratz, L.; Cozma, D.; Kovarova, M.; Harris, G.; Grinberg, A.; Yang, Y.; Hunter, A.G.; Tsokos, M.; Kelley, R.I.; Porter, F.D.
Lathosterolosis: an inborn error of human and murine cholesterol synthesis due to lathosterol 5-desaturase deficiency
Hum. Mol. Genet.
12
1631-1641
2003
Mus musculus
brenda
Lin, X.; Chen, Z.; Yue, P.; Averna, M.R.; Ostlund, R.E.; Watson, M.A.; Schonfeld, G.
A targeted apoB38.9 mutation in mice is associated with reduced hepatic cholesterol synthesis and enhanced lipid peroxidation
Am. J. Physiol. Gastrointest. Liver Physiol.
290
G1170-G1176
2006
Mus musculus (O88822), Mus musculus
brenda
Giera, M.; Renard, D.; Ploessl, F.; Bracher, F.
Lathosterol side chain amidesa new class of human lathosterol oxidase inhibitors
Steroids
73
299-308
2008
Homo sapiens
brenda
Rossi, M.; DArmiento, M.; Parisi, I.; Ferrari, P.; Hall, C.M.; Cervasio, M.; Rivasi, F.; Balli, F.; Vecchione, R.; Corso, G.; Andria, G.; Parenti, G.
Clinical phenotype of lathosterolosis
Am. J. Med. Genet. A
143A
2371-2381
2007
Homo sapiens
brenda
Nusblat, A.D.; Najle, S.R.; Tomazic, M.L.; Uttaro, A.D.; Nudel, C.B.
C-5(6) sterol desaturase from Tetrahymena thermophila: Gene identification and knockout, sequence analysis, and comparison to other C-5(6) sterol desaturases
Eukaryot. Cell
8
1287-1297
2009
Tetrahymena thermophila (C4PFX1), Tetrahymena thermophila, Tetrahymena thermophila CU428 (C4PFX1)
brenda
Acimovic, J.; Korosec, T.; Seliskar, M.; Bjorkhem, I.; Monostory, K.; Szabo, P.; Pascussi, J.M.; Belic, A.; Urleb, U.; Kocjan, D.; Rozman, D.
Inhibition of human sterol DELTA7-reductase and other postlanosterol enzymes by LK-980, a novel inhibitor of cholesterol synthesis
Drug Metab. Dispos.
39
39-46
2011
Homo sapiens
brenda
Jiang, X.S.; Backlund, P.S.; Wassif, C.A.; Yergey, A.L.; Porter, F.D.
Quantitative proteomics analysis of inborn errors of cholesterol synthesis: identification of altered metabolic pathways in DHCR7 and SC5D deficiency
Mol. Cell. Proteomics
9
1461-1475
2010
Mus musculus
brenda
Silvestro, D.; Andersen, T.; Schaller, H.; Jensen, P.
Plant sterol metabolism. delta7-Sterol-C5-desaturase (STE1/DWARF7), delta5,7-sterol-delta7-reductase (DWARF5) and delta24-sterol-delta24-reductase (DIMINUTO/DWARF1) show multiple subcellular localizations in Arabidopsis thaliana (Heynh) L
PLoS ONE
8
e56429
2013
Arabidopsis thaliana
brenda
Chau, A.; Gurnani, M.; Hawkinson, R.; Laverdiere, M.; Cacciapuoti, A.; McNicholas, P.
Inactivation of sterol DELTA5,6-desaturase attenuates virulence in Candida albicans
Antimicrob. Agents Chemother.
49
3646-3651
2005
Candida albicans (O93875), Candida albicans
brenda
Taton, M.; Rahier, A.
Plant sterol biosynthesis: Identification and characterization of higher plant DELTA7-sterol C5(6)-desaturase
Arch. Biochem. Biophys.
325
279-288
1996
Zea mays
brenda
Nishino, H.; Nakaya, J.; Nishi, S.; Kurosawa, T.; Ishibashi, T.
Temperature-induced differential kinetic properties between an initial burst and the following steady state in membrane-bound enzymes: studies on lathosterol 5-desaturase
Arch. Biochem. Biophys.
339
298-304
1997
Rattus norvegicus
brenda
Nishino, H.; Nishi, S.; Ishibashi, T.
Nine of ten histidine residues, located in three conserved clusters, are required for mammalian sterol C5-desaturase activity
Biomed. Res.
24
99-106
2003
Homo sapiens (O75845)
-
brenda
Arthington, B.A.; Bennett, L.G.; Skatrud, P.L.; Guynn, C.J.; Barbuch, R.J.; Ulbright, C.E.; Bard, M.
Cloning, disruption and sequence of the gene encoding yeast C-5 sterol desaturase
Gene
102
39-44
1991
Saccharomyces cerevisiae
brenda
Morio, F.; Pagniez, F.; Lacroix, C.; Miegeville, M.; Le pape, P.
Amino acid substitutions in the Candida albicans sterol DELTA5,6-desaturase (Erg3p) confer azole resistance: Characterization of two novel mutants with impaired virulence
J. Antimicrob. Chemother.
67
2131-2138
2012
Candida albicans (J7FE12)
brenda
Honjo, K.; Ishibashi, T.; Imai, Y.
Partial purification and characterization of lathosterol 5-desaturase from rat liver microsomes
J. Biochem.
97
955-959
1985
Rattus norvegicus
brenda
Takakuwa, Y.; Nishino, H.; Ishibe, Y.; Ishibashi, T.
Properties and kinetics of membrane-bound enzymes when both the enzyme and substrate are components of the same microsomal membrane. Studies on lathosterol 5-desaturase
J. Biol. Chem.
269
27889-27893
1994
Rattus norvegicus
brenda
Gachotte, D.; Meens, R.; Benveniste, P.
An Arabidopsis mutant deficient in sterol biosynthesis: heterologous complementation by ERG 3 encoding a DELTA7-sterol-C-5-desaturase from yeast
Plant J.
8
407-416
1995
Saccharomyces cerevisiae
brenda
Gachotte, D.; Husselstein, T.; Bard, M.; Lacroute, F.; Benveniste, P.
Isolation and characterization of an Arabidopsis thaliana cDNA encoding a DELTA7-sterol-C-5-desaturase by functional complementation of a defective yeast mutant
Plant J.
9
391-398
1996
Arabidopsis thaliana
brenda
Poklepovich, T.J.; Rinaldi, M.A.; Tomazic, M.L.; Favale, N.O.; Turkewitz, A.P.; Nudel, C.B.; Nusblat, A.D.
The cytochrome b5 dependent C-5(6) sterol desaturase DES5A from the endoplasmic reticulum of Tetrahymena thermophila complements ergosterol biosynthesis mutants in Saccharomyces cerevisiae
Steroids
77
1313-1320
2012
Tetrahymena thermophila (C4PFX1), Tetrahymena thermophila, Tetrahymena thermophila CU428 (C4PFX1)
brenda
Rybak, J.; Dickens, C.; Parker, J.; Caudle, K.; Manigaba, K.; Whaley, S.; Nishimoto, A.; Luna-Tapia, A.; Roy, S.; Zhang, Q.; Barker, K.; Palmer, G.; Sutter, T.; Homayouni, R.; Wiederhold, N.; Kelly, S.; Rogers, P.
Loss of C-5 sterol desaturase activity results in increased resistance to azole and echinocandin antifungals in a clinical isolate of candida parapsilosis
Antimicrob. Agents Chemother.
61
e00651-17
2017
Candida parapsilosis (A0A1L2BH72), Candida parapsilosis, Candida albicans (Q59VG6), Candida albicans, Candida parapsilosis CDC317 (A0A1L2BH72)
brenda
Makarova, A.M.; Pasta, S.; Watson, G.; Shackleton, C.; Epstein, E.H.
Attenuation of UVR-induced vitamin D3 synthesis in a mouse model deleted for keratinocyte lathosterol 5-desaturase
J. Steroid Biochem. Mol. Biol.
171
187-194
2017
Mus musculus, Mus musculus C57Bl/6N (JM8A3.N1)
brenda
Kamthan, A.; Kamthan, M.; Datta, A.
Expression of C-5 sterol desaturase from an edible mushroom in fisson yeast enhances its ethanol and thermotolerance
PLoS ONE
12
e0173381
2017
Flammulina velutipes (O13666), Flammulina velutipes
brenda