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4,4-dimethyl-5alpha-cholesta-7,14-dien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-7-en-3beta-ol + NADP+
4,4-dimethyl-5alpha-cholesta-8,12,24-trien-3beta-ol + NADPH + H+
4,4-dimethylzymosterol + NADP+
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH + H+
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
4,4-dimethyl-5alpha-cholesta-8,14-dien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8-en-3beta-ol + NADP+
4,4-dimethyl-5alpha-cholesta-8,14-dien-3beta-ol + NADPH + H+
4,4-dimethyl-5alpha-cholesta-8-en-3beta-ol + NADP+
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH + H+
4alpha-methyl-5alpha-ergosta-8,14,24(28)-trien-3beta-ol + NADPH + H+
4alpha-methylfecosterol + NADP+
4alpha-methyl-8,14,24(28)-ergostatrien-3beta-ol + NADPH
4alpha-methyl-8,24(28)-ergostadien-3beta-ol + NADP+
5alpha-cholesta-7,14-dien-3beta-ol + NADPH
5alpha-cholesta-7-en-3beta-ol + NADP+
-
43% activity compared with 4,4-dimethyl-5alpha-cholesta-8,14-dien-3beta-ol
-
-
?
5alpha-cholesta-8,14-dien-3beta-ol + NADPH
5alpha-cholesta-8-en-3beta-ol + NADP+
5alpha-cholesta-8,14-dien-3beta-ol + NADPH + H+
5alpha-cholesta-8-en-3beta-ol + NADP+
5alpha-ergosta-8,14,24(28)-trien-3beta-ol + NADPH
5alpha-ergosta-8,24(28)-dien-3beta-ol + NADP+
cholesta-8,14-dien-3beta-ol + NADPH
cholesta-8-en-3beta-ol + NADP+
dihydro-follicular fluid meiosis-activating sterol + NADPH + H+
dihydro-testis meiosis-activating sterol + NADP+
-
-
-
?
ergosta-8,14-dien-3beta-ol + NADPH
ergosta-8-en-3beta-ol + NADP+
-
DELTA8,14-sterol, ignosterol
-
-
?
follicular fluid meiosis-activating sterol + NADPH
testicular meiosis-activating sterol + NADP+
-
-
-
-
?
follicular fluid meiosis-activating sterol + NADPH + H+
testis meiosis-activating sterol + NADP+
-
-
-
?
isofucosterol + NADPH
sitosterol + NADP+
additional information
?
-
4,4-dimethyl-5alpha-cholesta-7,14-dien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-7-en-3beta-ol + NADP+
-
-
-
-
?
4,4-dimethyl-5alpha-cholesta-7,14-dien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-7-en-3beta-ol + NADP+
-
64% activity compared with 4,4-dimethyl-5alpha-cholesta-8,14-dien-3beta-ol
-
-
?
4,4-dimethyl-5alpha-cholesta-8,12,24-trien-3beta-ol + NADPH + H+
4,4-dimethylzymosterol + NADP+
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,12,24-trien-3beta-ol + NADPH + H+
4,4-dimethylzymosterol + NADP+
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
-
C29DELTA8,14,24
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
-
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
-
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
-
probably the natural substrate
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
-
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
-
probably the natural substrate
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH + H+
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH + H+
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14-dien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8-en-3beta-ol + NADP+
-
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14-dien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8-en-3beta-ol + NADP+
-
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14-dien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8-en-3beta-ol + NADP+
-
best substrate, probably natural substrate
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14-dien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8-en-3beta-ol + NADP+
-
presumably the natural substrate
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14-dien-3beta-ol + NADPH + H+
4,4-dimethyl-5alpha-cholesta-8-en-3beta-ol + NADP+
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14-dien-3beta-ol + NADPH + H+
4,4-dimethyl-5alpha-cholesta-8-en-3beta-ol + NADP+
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH + H+
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH + H+
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH + H+
-
-
-
?
4alpha-methyl-5alpha-ergosta-8,14,24(28)-trien-3beta-ol + NADPH + H+
4alpha-methylfecosterol + NADP+
-
-
-
-
?
4alpha-methyl-5alpha-ergosta-8,14,24(28)-trien-3beta-ol + NADPH + H+
4alpha-methylfecosterol + NADP+
-
step in the biosynthesis of the brassicasterol phytohormones, catalyzed by enzyme FK
-
-
?
4alpha-methyl-5alpha-ergosta-8,14,24(28)-trien-3beta-ol + NADPH + H+
4alpha-methylfecosterol + NADP+
-
-
-
-
?
4alpha-methyl-8,14,24(28)-ergostatrien-3beta-ol + NADPH
4alpha-methyl-8,24(28)-ergostadien-3beta-ol + NADP+
-
4alpha-methyl-5alpha-ergosta-8,14,24(28)-trien-3beta-ol
-
-
?
4alpha-methyl-8,14,24(28)-ergostatrien-3beta-ol + NADPH
4alpha-methyl-8,24(28)-ergostadien-3beta-ol + NADP+
-
4alpha-methyl-5alpha-ergosta-8,14,24(28)-trien-3beta-ol
-
-
?
5alpha-cholesta-8,14-dien-3beta-ol + NADPH
5alpha-cholesta-8-en-3beta-ol + NADP+
-
DELTA8,14-cholestadien-3beta-ol
-
-
?
5alpha-cholesta-8,14-dien-3beta-ol + NADPH
5alpha-cholesta-8-en-3beta-ol + NADP+
-
C27DELTA8,14
-
-
?
5alpha-cholesta-8,14-dien-3beta-ol + NADPH
5alpha-cholesta-8-en-3beta-ol + NADP+
-
DELTA8,14-cholestadien-3beta-ol
-
-
?
5alpha-cholesta-8,14-dien-3beta-ol + NADPH
5alpha-cholesta-8-en-3beta-ol + NADP+
-
-
-
?
5alpha-cholesta-8,14-dien-3beta-ol + NADPH
5alpha-cholesta-8-en-3beta-ol + NADP+
the substrate accumulates in patients suffering autosomal recessive HEM/Greenberg skeletal dysplasia
-
-
?
5alpha-cholesta-8,14-dien-3beta-ol + NADPH
5alpha-cholesta-8-en-3beta-ol + NADP+
-
DELTA8,14-cholestadien-3beta-ol
-
-
?
5alpha-cholesta-8,14-dien-3beta-ol + NADPH
5alpha-cholesta-8-en-3beta-ol + NADP+
-
53% activity compared with 4,4-dimethyl-5alpha-cholesta-8,14-dien-3beta-ol
-
-
?
5alpha-cholesta-8,14-dien-3beta-ol + NADPH
5alpha-cholesta-8-en-3beta-ol + NADP+
-
DELTA8,14-cholestadien-3beta-ol
-
-
?
5alpha-cholesta-8,14-dien-3beta-ol + NADPH + H+
5alpha-cholesta-8-en-3beta-ol + NADP+
-
LBR
-
-
?
5alpha-cholesta-8,14-dien-3beta-ol + NADPH + H+
5alpha-cholesta-8-en-3beta-ol + NADP+
-
TM7SF2
-
-
?
5alpha-cholesta-8,14-dien-3beta-ol + NADPH + H+
5alpha-cholesta-8-en-3beta-ol + NADP+
-
-
-
?
5alpha-cholesta-8,14-dien-3beta-ol + NADPH + H+
5alpha-cholesta-8-en-3beta-ol + NADP+
-
-
-
?
5alpha-ergosta-8,14,24(28)-trien-3beta-ol + NADPH
5alpha-ergosta-8,24(28)-dien-3beta-ol + NADP+
-
-
-
-
?
5alpha-ergosta-8,14,24(28)-trien-3beta-ol + NADPH
5alpha-ergosta-8,24(28)-dien-3beta-ol + NADP+
-
-
-
-
?
cholesta-8,14-dien-3beta-ol + NADPH
cholesta-8-en-3beta-ol + NADP+
-
-
-
-
?
cholesta-8,14-dien-3beta-ol + NADPH
cholesta-8-en-3beta-ol + NADP+
-
-
-
-
?
isofucosterol + NADPH
sitosterol + NADP+
-
-
-
-
?
isofucosterol + NADPH
sitosterol + NADP+
-
step in the biosynthesis of the brassicasterol phytohormones, catalyzed by bifunctinal enzyme DIM1/CBB1/DWF1
-
-
?
additional information
?
-
-
brassicasterols have regulatory function in the plants concerning growth and development, the enzyme is up-regulated by several growth-promoting hormones including brassinolide and auxin, overview
-
-
?
additional information
?
-
-
enzyme acts on a range of steroids with a 14(15)-double bond
-
-
?
additional information
?
-
-
14-reductase has a important regulatory role in the overall cholesterol synthetic pathway
-
-
?
additional information
?
-
-
important essential enzyme of cholesterol biosynthesis from lanosterol
-
-
?
additional information
?
-
-
enzyme plays a key role in sterol biosynthesis, sterol content in vivo
-
-
?
additional information
?
-
-
substrate specificity
-
-
?
additional information
?
-
-
enzyme acts on a range of steroids with a 14(15)-double bond
-
-
?
additional information
?
-
-
enzyme involved in sterol, phytosterol, biosynthesis, DELTA8,14-sterols are intermediates in the biosynthesis of higher plant sterols, phytosterols
-
-
?
additional information
?
-
-
enzyme acts on a range of steroids with a 14(15)-double bond
-
-
?
additional information
?
-
-
14-reductase has a important regulatory role in the overall cholesterol synthetic pathway
-
-
?
additional information
?
-
-
important essential enzyme of cholesterol biosynthesis from lanosterol
-
-
?
additional information
?
-
-
enzyme plays a key role in sterol biosynthesis, sterol content in vivo
-
-
?
additional information
?
-
enzyme-deficiency causes the lethal autosomal recessive HEM/Greenberg skeletal dysplasia, characterized by short limbs, fetal hydrops, abnormal chondro-osseous calcification
-
-
?
additional information
?
-
enzyme-deficiency causes the lethal autosomal recessive HEM/Greenberg skeletal dysplasia, characterized by short limbs, fetal hydrops, abnormal chondro-osseous calcification
-
-
?
additional information
?
-
-
enzyme-deficiency causes the lethal autosomal recessive HEM/Greenberg skeletal dysplasia, characterized by short limbs, fetal hydrops, abnormal chondro-osseous calcification
-
-
?
additional information
?
-
-
C14SR is encoded by the TM7SF2 gene. TM7SF2 but not LBR, gene expression is regulated by cell sterol levels in HepG2 (human) and H-35 (rat) hepatoma cells. Primary role of C14SR in human cholesterol biosynthesis
-
-
?
additional information
?
-
-
role of LBR in the cholesterol biosynthesis pathway is unclear
-
-
?
additional information
?
-
enzyme MaSR1 can reduce the double bond of a cholesterol biosynthetic intermediate analogously to the human enzyme
-
-
?
additional information
?
-
-
enzyme MaSR1 can reduce the double bond of a cholesterol biosynthetic intermediate analogously to the human enzyme
-
-
?
additional information
?
-
enzyme MaSR1 can reduce the double bond of a cholesterol biosynthetic intermediate analogously to the human enzyme
-
-
?
additional information
?
-
-
enzyme MaSR1 can reduce the double bond of a cholesterol biosynthetic intermediate analogously to the human enzyme
-
-
?
additional information
?
-
-
enzyme plays a key role in sterol biosynthesis
-
-
?
additional information
?
-
-
enzyme plays a key role in sterol biosynthesis, sterol content in vivo
-
-
?
additional information
?
-
-
the charge and geometry given via residues E233, R235, D289, K351, R458, and R461 is important for enzyme activity
-
-
?
additional information
?
-
-
substrate specificity
-
-
?
additional information
?
-
-
enzyme acts on a range of steroids with a 14(15)-double bond
-
-
?
additional information
?
-
-
enzyme acts on a range of steroids with a 14(15)-double bond
-
-
?
additional information
?
-
-
reduction of 14-double bond of conjugated DELTA8,14-diene sterols and DELTA7,14-diene sterols
-
-
?
additional information
?
-
-
sterol substrate specificity study
-
-
?
additional information
?
-
-
anaerobic conditions yield maximal rates of reduction
-
-
?
additional information
?
-
-
4-nor-methyldiene sterols, 5alpha-cholesta-8,14-dien-3beta-ol, and 5alpha-cholesta-7,14-dien-3beta-ol are reduced at about 50% of the rate of the corresponding 4-gem-dimethyldiene sterols, for both the C27-sterol and C29-sterol, the higher reaction rate of the 8,14-diene system is consistently observed over the isomeric 7,14-diene
-
-
?
additional information
?
-
-
catalyzes anaerobically NADPH-dependent reduction of 14-double bond of 8,14-diene or 7,14-diene sterols that are sterol intermediates formed during C-32 demethylation in cholesterol biosynthesis from lanosterol in mammals
-
-
?
additional information
?
-
-
catalyzes anaerobically NADPH-dependent reduction of 14-double bond of 8,14-diene or 7,14-diene sterols that are sterol intermediates formed during C-32 demethylation in cholesterol biosynthesis from lanosterol in mammals
-
-
?
additional information
?
-
-
reduction of 14-double bond of sterol-conjugated dienes
-
-
?
additional information
?
-
-
14-reductase has a important regulatory role in the overall cholesterol synthetic pathway
-
-
?
additional information
?
-
-
microsomal enzyme of cholesterol biosynthesis from lanosterol
-
-
?
additional information
?
-
-
important essential enzyme of cholesterol biosynthesis from lanosterol
-
-
?
additional information
?
-
-
enzyme acts on a range of steroids with a 14(15)-double bond
-
-
?
additional information
?
-
-
reduction of DELTA8,14-intermediate in ergosterol biosynthetic pathway to DELTA8-intermediate occurs by the trans addition of two hydrogens to the 14alpha- and 15beta-positions
-
-
?
additional information
?
-
-
enzyme of biosynthetic pathways of ergosterol, major sterol in yeast, and cholesterol including a DELTA8,14-sterol intermediate as result of demethylation of lanosterol at C-14
-
-
?
additional information
?
-
-
essential enzyme of ergosterol, yeast sterol, biosynthesis from lanosterol
-
-
?
additional information
?
-
-
essential enzyme of ergosterol, yeast sterol, biosynthesis from lanosterol
-
-
?
additional information
?
-
-
enzyme acts on a range of steroids with a 14(15)-double bond
-
-
?
additional information
?
-
-
reduction of DELTA8,14-intermediate in ergosterol biosynthetic pathway to DELTA8-intermediate occurs by the trans addition of two hydrogens to the 14alpha- and 15beta-positions
-
-
?
additional information
?
-
-
enzyme of biosynthetic pathways of ergosterol, major sterol in yeast, and cholesterol including a DELTA8,14-sterol intermediate as result of demethylation of lanosterol at C-14
-
-
?
additional information
?
-
-
essential enzyme of ergosterol, yeast sterol, biosynthesis from lanosterol
-
-
?
additional information
?
-
-
enzyme is involved in de novo synthesis of progesterone from lanosterol via cholesterol, pathway overview
-
-
?
additional information
?
-
-
substrate specificity
-
-
?
additional information
?
-
-
enzyme acts on a range of steroids with a 14(15)-double bond
-
-
?
additional information
?
-
-
DELTA8,14 derivatives are the only substrates to be transformed under normal biosynthetic conditions
-
-
?
additional information
?
-
-
no activity with 5alpha-cholesta-7,14-dien-3beta-ol, 7,14-cholestadien-3beta-ol or 5alpha-cholest-14-en-3beta-ol, 14-cholesten-3beta-ol
-
-
?
additional information
?
-
-
important roles of 4alpha-methyl group and side-chain substitution for recognition but not for catalysis, presence of DELTA8(9) unsaturation is compulsory for catalysis
-
-
?
additional information
?
-
-
enzyme involved in sterol, phytosterol, biosynthesis, DELTA8,14-sterols are intermediates in the biosynthesis of higher plant sterols, phytosterols
-
-
?
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH + H+
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
4,4-dimethyl-5alpha-cholesta-8,14-dien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8-en-3beta-ol + NADP+
-
presumably the natural substrate
-
-
?
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH + H+
4alpha-methyl-5alpha-ergosta-8,14,24(28)-trien-3beta-ol + NADPH + H+
4alpha-methylfecosterol + NADP+
-
step in the biosynthesis of the brassicasterol phytohormones, catalyzed by enzyme FK
-
-
?
4alpha-methyl-8,14,24(28)-ergostatrien-3beta-ol + NADPH
4alpha-methyl-8,24(28)-ergostadien-3beta-ol + NADP+
5alpha-cholesta-8,14-dien-3beta-ol + NADPH
5alpha-cholesta-8-en-3beta-ol + NADP+
the substrate accumulates in patients suffering autosomal recessive HEM/Greenberg skeletal dysplasia
-
-
?
follicular fluid meiosis-activating sterol + NADPH
testicular meiosis-activating sterol + NADP+
-
-
-
-
?
isofucosterol + NADPH
sitosterol + NADP+
-
step in the biosynthesis of the brassicasterol phytohormones, catalyzed by bifunctinal enzyme DIM1/CBB1/DWF1
-
-
?
additional information
?
-
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
-
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
-
probably the natural substrate
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
-
probably the natural substrate
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH + H+
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH + H+
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH + H+
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH + H+
-
-
-
?
4,4-dimethyl-5alpha-cholesta-8,24-dien-3beta-ol + NADP+
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + NADPH + H+
-
-
-
?
4alpha-methyl-8,14,24(28)-ergostatrien-3beta-ol + NADPH
4alpha-methyl-8,24(28)-ergostadien-3beta-ol + NADP+
-
4alpha-methyl-5alpha-ergosta-8,14,24(28)-trien-3beta-ol
-
-
?
4alpha-methyl-8,14,24(28)-ergostatrien-3beta-ol + NADPH
4alpha-methyl-8,24(28)-ergostadien-3beta-ol + NADP+
-
4alpha-methyl-5alpha-ergosta-8,14,24(28)-trien-3beta-ol
-
-
?
additional information
?
-
-
brassicasterols have regulatory function in the plants concerning growth and development, the enzyme is up-regulated by several growth-promoting hormones including brassinolide and auxin, overview
-
-
?
additional information
?
-
-
14-reductase has a important regulatory role in the overall cholesterol synthetic pathway
-
-
?
additional information
?
-
-
important essential enzyme of cholesterol biosynthesis from lanosterol
-
-
?
additional information
?
-
-
enzyme plays a key role in sterol biosynthesis, sterol content in vivo
-
-
?
additional information
?
-
-
enzyme involved in sterol, phytosterol, biosynthesis, DELTA8,14-sterols are intermediates in the biosynthesis of higher plant sterols, phytosterols
-
-
?
additional information
?
-
-
14-reductase has a important regulatory role in the overall cholesterol synthetic pathway
-
-
?
additional information
?
-
-
important essential enzyme of cholesterol biosynthesis from lanosterol
-
-
?
additional information
?
-
-
enzyme plays a key role in sterol biosynthesis, sterol content in vivo
-
-
?
additional information
?
-
enzyme-deficiency causes the lethal autosomal recessive HEM/Greenberg skeletal dysplasia, characterized by short limbs, fetal hydrops, abnormal chondro-osseous calcification
-
-
?
additional information
?
-
enzyme-deficiency causes the lethal autosomal recessive HEM/Greenberg skeletal dysplasia, characterized by short limbs, fetal hydrops, abnormal chondro-osseous calcification
-
-
?
additional information
?
-
-
enzyme-deficiency causes the lethal autosomal recessive HEM/Greenberg skeletal dysplasia, characterized by short limbs, fetal hydrops, abnormal chondro-osseous calcification
-
-
?
additional information
?
-
-
C14SR is encoded by the TM7SF2 gene. TM7SF2 but not LBR, gene expression is regulated by cell sterol levels in HepG2 (human) and H-35 (rat) hepatoma cells. Primary role of C14SR in human cholesterol biosynthesis
-
-
?
additional information
?
-
-
role of LBR in the cholesterol biosynthesis pathway is unclear
-
-
?
additional information
?
-
-
enzyme plays a key role in sterol biosynthesis
-
-
?
additional information
?
-
-
enzyme plays a key role in sterol biosynthesis, sterol content in vivo
-
-
?
additional information
?
-
-
catalyzes anaerobically NADPH-dependent reduction of 14-double bond of 8,14-diene or 7,14-diene sterols that are sterol intermediates formed during C-32 demethylation in cholesterol biosynthesis from lanosterol in mammals
-
-
?
additional information
?
-
-
catalyzes anaerobically NADPH-dependent reduction of 14-double bond of 8,14-diene or 7,14-diene sterols that are sterol intermediates formed during C-32 demethylation in cholesterol biosynthesis from lanosterol in mammals
-
-
?
additional information
?
-
-
reduction of 14-double bond of sterol-conjugated dienes
-
-
?
additional information
?
-
-
14-reductase has a important regulatory role in the overall cholesterol synthetic pathway
-
-
?
additional information
?
-
-
microsomal enzyme of cholesterol biosynthesis from lanosterol
-
-
?
additional information
?
-
-
important essential enzyme of cholesterol biosynthesis from lanosterol
-
-
?
additional information
?
-
-
reduction of DELTA8,14-intermediate in ergosterol biosynthetic pathway to DELTA8-intermediate occurs by the trans addition of two hydrogens to the 14alpha- and 15beta-positions
-
-
?
additional information
?
-
-
enzyme of biosynthetic pathways of ergosterol, major sterol in yeast, and cholesterol including a DELTA8,14-sterol intermediate as result of demethylation of lanosterol at C-14
-
-
?
additional information
?
-
-
essential enzyme of ergosterol, yeast sterol, biosynthesis from lanosterol
-
-
?
additional information
?
-
-
essential enzyme of ergosterol, yeast sterol, biosynthesis from lanosterol
-
-
?
additional information
?
-
-
reduction of DELTA8,14-intermediate in ergosterol biosynthetic pathway to DELTA8-intermediate occurs by the trans addition of two hydrogens to the 14alpha- and 15beta-positions
-
-
?
additional information
?
-
-
enzyme of biosynthetic pathways of ergosterol, major sterol in yeast, and cholesterol including a DELTA8,14-sterol intermediate as result of demethylation of lanosterol at C-14
-
-
?
additional information
?
-
-
essential enzyme of ergosterol, yeast sterol, biosynthesis from lanosterol
-
-
?
additional information
?
-
-
enzyme is involved in de novo synthesis of progesterone from lanosterol via cholesterol, pathway overview
-
-
?
additional information
?
-
-
DELTA8,14 derivatives are the only substrates to be transformed under normal biosynthetic conditions
-
-
?
additional information
?
-
-
enzyme involved in sterol, phytosterol, biosynthesis, DELTA8,14-sterols are intermediates in the biosynthesis of higher plant sterols, phytosterols
-
-
?
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15-aza-24-methylene-D-homocholestadiene-3beta-ol
2-amino-nonyl-6-methoxyl-tetralin muriate
-
-
25-hydroxy-cholesterol
-
IC50: 0.45 mM
3beta-(2-(diethylamino)ethoxy)androst-5-en-17-one
-
U18666A, IC50: 0.004 mM
4-(3-[[1-(4-bromophenyl)piperidin-4-yl](methyl)amino]-1-methoxypropyl)benzonitrile
-
using a genetic aproach in Saccharomyces cerevisiae it is shown that ERG24 is the target enzyme of compound 4-(3-[[1-(4-bromophenyl)piperidin-4-yl](methyl)amino]-1-methoxypropyl)benzonitrile
8-aza-4alpha,10-dimethyl-trans-decal-3beta-ol
A25822B
-
competitive inhibitor
AY9944-A-7
-
enzyme-specific inhibitor, leads to apoptosis in vivo at high concentrations in cumulus cells of oocytes
brefeldin
-
inhibition of growth of mutant strains NJ25, NJ50, NJ51, and NJ55, not of wild-type strain and mutant NJ25, overview
cerulenin
-
inhibition of growth of mutant strains NJ25, NJ50, NJ51, and NJ55, not of wild-type strain and mutant NJ25, overview
cholesterol
-
enzyme suppressed by feeding 5% cholesterol, 70% decrease in enzyme activity
clotrimazole
-
inhibition of growth of wild-type and mutant strains NJ25, NJ50, NJ51, and NJ55, overview
Detergents
-
enzyme solubilization by detergents: inactivation
fluconazole
-
inhibition of growth of wild-type and mutant strain NJ25, only slightly of mutant strains NJ50, NJ51, and NJ55, overview
fluphenazine
-
inhibition of growth of mutant strains NJ25, NJ50, NJ51, and NJ55, not of wild-type strain and mutant NJ25, overview
HgCl2
-
3 mM: more than 99% inhibition, inhibition negligible in the presence of 10 mM glutathione
itraconazole
-
inhibition of growth of wild-type and mutant strain NJ25, only slightly of mutant strains NJ50, NJ51, and NJ55, overview
ketoconazole
-
inhibition of growth of wild-type and mutant strains NJ25, NJ50, NJ51, and NJ55, overview
miconazole
-
IC50: 0.15 mM
N(1,5,9-trimethyldecyl)-4alpha,10-dimethyl-8-aza-trans-decal-3beta-ol
N-alkyl morpholine derivatives
-
fungicides
N-alkyl-8-aza-4alpha,10-dimethyl-trans-decal-3beta-ol
-
N-Benzyl-8-aza-4alpha,10-dimethyl-trans-decal-3beta-ol
N-dodecyl-8-aza-4alpha,10-dimethyl-trans-decal-3beta-ol
N-ethylmaleimide
-
5 mM: 53% inhibition, inhibition negligible in the presence of 10 mM glutathione
N-substituted 8-azadecalins
-
potent inhibitors
N-[(3)-4-tert-butylphenyl-(2-methyl)propyl]-8-aza-4alpha,10-dimethyl-trans-decal-3beta-ol
NADP+
-
in the absence of NADPH-regenerating system, Ki: 0.45 mM; no inhibition by NAD+
Nystatin
-
inhibition of growth of wild-type and mutant strain NJ25, not of mutants strains NJ50, NJ51, and NJ55, overview
Phospholipase A2
-
very high inhibition by very small concentrations, activity not restored by addition of phospholipids or any components presumed to be released from treated microsomes. Various concentration ranges of potential cofactors including FMN, FAD, AMP, ADP, ATP, coenzyme Q, Coenzyme A, and hemin, plus heat-treated microsomes, and lipid extracts of microsomes does not restore microsomal 14-reductase of phospholipase A2-treated microsomes
-
sulfhydryl-binding agents
-
-
-
terbinafine
-
inhibition of growth of mutant strains NJ25, NJ50, NJ51, and NJ55, not of wild-type strain and mutant NJ25, overview
trans-1,4-bis(2-chlorobenzylaminomethyl)cyclohexane dihydrochloride
Triparanol
-
4-chloro-alpha-[4-[2-diethylaminoethoxy]phenyl]-alpha-(4-methylphenyl)benze-methanol, slight inhibition
Tris-HCl
-
Tris-HCl buffer, 28% inhibition, not restored by addition of KCl at various concentrations
Tween 80
-
concentrations of Tween 80 higher than 1.5 g/l: inhibitory effect
15-aza-24-methylene-D-homocholestadiene-3beta-ol
-
A25822B, extremely potent inhibitor
15-aza-24-methylene-D-homocholestadiene-3beta-ol
-
A25822B, extremely potent inhibitor
15-azasterol
-
antimycotic agent, IC50: 0.00003 mM
15-azasterol
-
15-aza-24-methylene-D-homocholestadiene; potent antimycotic agent, designated A258223, non-competitively inhibition at very low concentrations, Ki: 0.000002 mM
15-azasterol
-
antimycotic agent, IC50: 0.00003 mM
8-aza-4alpha,10-dimethyl-trans-decal-3beta-ol
-
extremely potent inhibitor
8-aza-4alpha,10-dimethyl-trans-decal-3beta-ol
-
extremely potent inhibitor
Ca2+
-
slight inhibition of growth of the wild-type strain, increased inhibition of growth of erg24 mutant strains
Ca2+
-
4 mM: very slight, 3.5% inhibition
cyanide
-
no inhibition
cyanide
-
cyanide ion, membrane-bound enzyme, modest inhibition at high concentrations, greater than 1 mM, partial purified enzyme, sodium cyanide, no or very poor inhibition, 10 mM: 3.5% inhibition
cycloheximide
-
inhibition of growth of mutant strains NJ25, NJ50, NJ51, and NJ55, not of wild-type strain and mutant NJ25, overview
cycloheximide
-
the 2.5fold increase in enzymic activity due to the diurnal rhythm is completely abolished by cycloheximide
fenpropidin
-
-
fenpropidin
-
fungicide, very potent inhibitor; IC50: 0.0018 mM; N-[3-(p-tert-butylphenyl)-2-methylpropyl]-piperidine
fenpropidin
-
fungicide, very potent inhibitor; N-[3-(p-tert-butylphenyl)-2-methylpropyl]-piperidine
fenpropidin
-
good inhibitor, IC50: 0.003 mM
fenpropimorph
-
impairs cell expansion and affects gene expression in vivo, the effects cannot be reversed by addition of exogenous brassicasterols, changes sterol composition, phenotype is similar to fk-mutant plants, molecular mechanism
fenpropimorph
-
inhibition of growth of wild-type and mutant strains NJ25, NJ50, NJ51, and NJ55, overview
fenpropimorph
-
fungicide, very potent inhibitor; IC50: 0.0023 mM; N-[3-(p-tert-butylphenyl)-2-methylpropyl]-cis-2,6-dimethylmorpholine
fenpropimorph
-
fungicide, very potent inhibitor; N-[3-(p-tert-butylphenyl)-2-methylpropyl]-cis-2,6-dimethylmorpholine
fenpropimorph
-
very good inhibitor, IC50: 0.0008 mM
Mg2+
-
4 mM: slight, 6% inhibition
N(1,5,9-trimethyldecyl)-4alpha,10-dimethyl-8-aza-trans-decal-3beta-ol
-
extremely potent inhibitor
N(1,5,9-trimethyldecyl)-4alpha,10-dimethyl-8-aza-trans-decal-3beta-ol
-
extremely potent inhibitor
N-alkyl-8-aza-4alpha,10-dimethyl-trans-decal-3beta-ol
-
strong inhibition
-
N-alkyl-8-aza-4alpha,10-dimethyl-trans-decal-3beta-ol
-
strong inhibition
-
N-Benzyl-8-aza-4alpha,10-dimethyl-trans-decal-3beta-ol
-
extremely potent inhibitor
N-Benzyl-8-aza-4alpha,10-dimethyl-trans-decal-3beta-ol
-
extremely potent inhibitor
N-dodecyl-8-aza-4alpha,10-dimethyl-trans-decal-3beta-ol
-
extremely potent inhibitor
N-dodecyl-8-aza-4alpha,10-dimethyl-trans-decal-3beta-ol
-
extremely potent inhibitor
N-[(3)-4-tert-butylphenyl-(2-methyl)propyl]-8-aza-4alpha,10-dimethyl-trans-decal-3beta-ol
-
extremely potent inhibitor
N-[(3)-4-tert-butylphenyl-(2-methyl)propyl]-8-aza-4alpha,10-dimethyl-trans-decal-3beta-ol
-
extremely potent inhibitor
trans-1,4-bis(2-chlorobenzylaminomethyl)cyclohexane dihydrochloride
-
AY-9944, strong inhibition, 0.0003 mM: 50% inhibition, 0.0005 mM: 65% inhibition
trans-1,4-bis(2-chlorobenzylaminomethyl)cyclohexane dihydrochloride
-
0.01% AY-9944: suppression and inhibition; AY-9944, competitive inhibitor, strong inhibition, Ki: 0.00026; AY-9944, direct inhibitory effects of sterol 14-reductase; hepatocyte, IC50: 0.00025 mM, microsomes, IC50: 0.0003 mM
trans-1,4-bis(2-chlorobenzylaminomethyl)cyclohexane dihydrochloride
-
AY 9944, very slight inhibition, IC50: 0.04 mM
tridemorph
-
inhibition of growth of wild-type and mutant strains NJ25, NJ50, NJ51, and NJ55, overview
tridemorph
-
2,6-dimethyl-N-tridecylmorpholine; fungicide; slight inhibitor, IC50: 0.098 mM
tridemorph
-
2,6-dimethyl-N-tridecylmorpholine; fungicide
tridemorph
-
slight inhibition, IC50: 0.025
additional information
-
inhibition by a series of ammonium-ion-containing fungicides
-
additional information
-
no direct inhibition by 3beta-hydroxy-4,4-dimethyl-5alpha-cholest-8(14)-en-15-one, even when concentration is increased to level of Km for substrate; no inhibition by trypsin
-
additional information
-
kinetic pattern of inhibition; no inhibition by lovastatin, mevalonolactone, Squalestatin 1, (E)-N-ethyl-N-(6,6-dimethyl-2-hepten-4-ynyl)-3-((3,3'-bithiophen-5-yl)methoxy)benzenemethanamine, NB-598
-
additional information
-
no inhibition by ergosterol, up to 0.2 mM
-
additional information
-
inhibition by a series of ammonium-ion-containing fungicides; no inhibition by 4alpha,10-dimethyl-trans-decal-3beta-ol; no inhibition by menadione or 10 mM NAD+
-
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Acute Kidney Injury
The Tm7sf2 Gene Deficiency Protects Mice against Endotoxin-Induced Acute Kidney Injury.
Adenoma
Differential gene expression profiling of aggressive and nonaggressive follicular carcinomas.
Alzheimer Disease
Quantitation of glycogen synthase kinase-3 sensitive proteins in neuronal membrane rafts.
Bone Diseases, Developmental
A new case of Greenberg dysplasia and literature review suggest that Greenberg dysplasia, dappled diaphyseal dysplasia, and Astley-Kendall dysplasia are allelic disorders.
Breast Neoplasms
Spatiotemporal Mislocalization of Nuclear Membrane-Associated Proteins in ?-Irradiation-Induced Senescent Cells.
Breast Neoplasms
The clinicopathological significance of lamin A/C, lamin B1 and lamin B receptor mRNA expression in human breast cancer.
Candidiasis
ERG2 and ERG24 Are Required for Normal Vacuolar Physiology as Well as Candida albicans Pathogenicity in a Murine Model of Disseminated but Not Vaginal Candidiasis.
Carcinoma
Differential gene expression profiling of aggressive and nonaggressive follicular carcinomas.
Carcinoma
Distinct cellular responses to replication stress leading to apoptosis or senescence.
Carcinoma
Spatiotemporal Mislocalization of Nuclear Membrane-Associated Proteins in ?-Irradiation-Induced Senescent Cells.
Chondrodysplasia Punctata
Genetic disorders of cholesterol biosynthesis in mice and humans.
Colonic Neoplasms
Comprehensive analysis of metastasis-related genes reveals a gene signature predicting the survival of colon cancer patients.
delta14-sterol reductase deficiency
Autosomal recessive HEM/Greenberg skeletal dysplasia is caused by 3 beta-hydroxysterol delta 14-reductase deficiency due to mutations in the lamin B receptor gene.
delta14-sterol reductase deficiency
HEM dysplasia and ichthyosis are likely laminopathies and not due to 3beta-hydroxysterol Delta14-reductase deficiency.
delta14-sterol reductase deficiency
Impaired cell proliferation in regenerating liver of 3 ?-hydroxysterol ?14-reductase (TM7SF2) knock-out mice.
Herpes Simplex
A three-residue signal confers localization of a reporter protein in the inner nuclear membrane.
Herpes Simplex
Fate of the inner nuclear membrane protein lamin B receptor and nuclear lamins in herpes simplex virus type 1 infection.
Ichthyosis
HEM dysplasia and ichthyosis are likely laminopathies and not due to 3beta-hydroxysterol Delta14-reductase deficiency.
Ichthyosis
Mutations at the mouse ichthyosis locus are within the lamin B receptor gene: a single gene model for human Pelger-Huët anomaly.
Infections
Characterization of lamin B receptor of Sf9 cells and its fate during Autographa californica nucleopolyhedrovirus infection.
Infections
Fate of the inner nuclear membrane protein lamin B receptor and nuclear lamins in herpes simplex virus type 1 infection.
Insulin Resistance
Tm7sf2 Disruption Alters Radial Gene Positioning in Mouse Liver Leading to Metabolic Defects and Diabetes Characteristics.
Insulin Resistance
Tm7sf2 gene promotes adipocyte differentiation of mouse embryonic fibroblasts and improves insulin sensitivity.
Laminopathies
HEM dysplasia and ichthyosis are likely laminopathies and not due to 3beta-hydroxysterol Delta14-reductase deficiency.
Laminopathies
LMNA, ZMPSTE24, and LBR are not mutated in scleroderma.
Leukemia
Antinuclear antibodies as ancillary markers in primary biliary cirrhosis.
Leukemia
Molecular diagnostics of primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Autoantibodies from patients with primary biliary cirrhosis recognize a region within the nucleoplasmic domain of inner nuclear membrane protein LBR.
Liver Cirrhosis, Biliary
Identification and characterization of autoantibodies against the nuclear envelope lamin B receptor from patients with primary biliary cirrhosis.
Melanoma
Discovery of key genes as novel biomarkers specifically associated with HPV-negative cervical cancer.
Metabolism, Inborn Errors
Genetic disorders of cholesterol biosynthesis in mice and humans.
Mevalonate Kinase Deficiency
Genetic disorders of cholesterol biosynthesis in mice and humans.
Neoplasms
Candidate diagnostic markers and tumor suppressor genes for adrenocortical carcinoma by expression profile of genes on chromosome 11q13.
Neoplasms
Differential gene expression profiling of aggressive and nonaggressive follicular carcinomas.
Neoplasms
Loss of lamin B receptor is necessary to induce cellular senescence.
Osteosarcoma
Spatiotemporal Mislocalization of Nuclear Membrane-Associated Proteins in ?-Irradiation-Induced Senescent Cells.
Papilloma
The loss of Tm7sf gene accelerates skin papilloma formation in mice.
Pelger-Huet Anomaly
Alterations in nuclear structure promote lupus autoimmunity in a mouse model.
Pelger-Huet Anomaly
Mouse neutrophils lacking lamin B-receptor expression exhibit aberrant development and lack critical functional responses.
Pelger-Huet Anomaly
Mutations at the mouse ichthyosis locus are within the lamin B receptor gene: a single gene model for human Pelger-Huët anomaly.
Renal Insufficiency
The Tm7sf2 Gene Deficiency Protects Mice against Endotoxin-Induced Acute Kidney Injury.
Scleroderma, Systemic
LMNA, ZMPSTE24, and LBR are not mutated in scleroderma.
Uterine Cervical Neoplasms
Discovery of key genes as novel biomarkers specifically associated with HPV-negative cervical cancer.
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evolution
the enzyme belongs to the sterol reductase family
evolution
-
the enzyme belongs to the sterol reductase family
-
malfunction
-
FgERG24A and FgERG24B are deleted in Fusarium graminearum. Compared to the wild-type strain HN9-1, FgERG24A and FgERG24B deletion mutants do not show recognizable phenotypic changes in mycelial growth on potato dextrose agar or in virulence on wheat heads. FgERG24B deletion mutants exhibit significantly increased sensitivity to amine fungicides, including tridemorph, fenpropidin and spiroxamine. FgERG24A deletion mutants do not show changed sensitivity to any amine tested. The resistance of the FgERG24B deletion mutant to amines is restored by genetic complementation of the mutant with wild-type FgERG24B
malfunction
Arabidopsis thaliana weak mutant allele fk-J3158, impaired in the FACKEL (FK) gene, which encodes the C-14 reductase, has a long life cycle and delayed flowering time in different photoperiods. Enzyme overexpression lines display an earlier flowering phenotype than that of the wild-type, flowering time of fk-J3158 flc double mutant is significantly earlier than that of fk-J3158 under the long-day conditions. These processes might be independent of the downstream brassinosteroid pathway and the autonomous pathway. The fk-J3158 plants are more sensitive than wild-type in reducing the bolting days and total leaf number under gibberellic acid treatment. Enzyme mutation leads to an absence of endogenous gibberellic acids in fk-J3158 and FK gene expression also affected under gibberellic acid and paclobutrazol treatment. The delayed flowering time of fk-J3158 is rescued by a 3-week vernalization treatment, and the expression of flowering locus C C (FLC) is accordingly downregulated in fk-J3158. Flowering time of fk-J3158 flc double mutant is significantly earlier than that of fk-J3158 under the long-day conditions. Phenotypes, detailed overview
malfunction
Tm7sf2 deficiency during liver regeneration alters lipid metabolism and generates a stress condition, which, in turn, transiently unbalances hepatocytes cell cycle progression. Tm7sf2 knockout mice show no alteration in cholesterol content, but accumulation and delayed catabolism of hepatic triglycerides is observed, resulting in persistent steatosis at all times post hepatectomy. Delayed cell cycle progression to the G1/S phase is observed in Tm7sf2 knockout mice, resulting in reduced cell division at the time points examined associated to abnormal endoplasmic reticulum stress response, leading to alteration in p53 content and, consequently, induction of p21 expression in Tm7sf2 knockout mice. Tm7sf2 knockout mice exhibit a high-degree of transient hepatic steatosis during liver regeneration after partial hepatectomy. Hepatocytes from Tm7sf2 KO mice have a defective progression through the G1/S phase, associated to delayed and/or reduced expression of central regulators of the cell cycle such as cyclin D1, cyclin A, cyclin E1 and CDK4
malfunction
inactivation of both homologs of Erg24 results in decreased hyphal growth and virulence, leads to the disruption of ion homeostasis and affects ergosterol biosynthesis. Loss of one homolog of Erg24 impairs hyphal growth, conidiation, and virulence but has no effect on ergosterol biosynthesis
malfunction
-
inactivation of both homologs of Erg24 results in decreased hyphal growth and virulence, leads to the disruption of ion homeostasis and affects ergosterol biosynthesis. Loss of one homolog of Erg24 impairs hyphal growth, conidiation, and virulence but has no effect on ergosterol biosynthesis
-
metabolism
-
an inverse upregulation of lanosterol 14alpha-demethylase, CYP51, and downregulation of 14-SR expression under luteinizing hormone/follicle stimulating hormone stimulation functions as the machinery for FF-MAS accumulation in preovulatory follicles prior to ovulation in the rabbit, overview
metabolism
the enzyme is involved in the cholesterol biosynthesis pathway
metabolism
-
the enzyme is involved in the cholesterol biosynthesis pathway
-
physiological function
-
FgERG24B controls the intrinsic resistance of Fusarium graminearum to amines
physiological function
the enzyme catalyzes the reduction of C14-unsaturated sterols during cholesterol biosynthesis from lanosterol. Role of C14-SR in vivo during cell proliferation by evaluating liver regeneration in Tm7sf2 knockout and wild-type mice, overview
physiological function
the enzyme is involved in sterol biosynthesis, but is also involved in the flowering of Arabidopsis mainly via the gibberellin pathway and vernalization pathway. This function of the enzyme is partially dependent on the FLOWERING LOCUS C (FLC)
physiological function
both erg24A and erg24B homologs in combination, but not individuall, are essential for the viability of Aspergillus fumigatus. The enzyme plays a critical role in ergosterol biosynthesis and ion homeostasis
physiological function
erg24A and erg24B homologs in combination, but not individually, are essential for the viability of Aspergillus fumigatus. The enzyme plays a critical role in ergosterol biosynthesis and ion homeostasis
physiological function
-
erg24A and erg24B homologs in combination, but not individually, are essential for the viability of Aspergillus fumigatus. The enzyme plays a critical role in ergosterol biosynthesis and ion homeostasis
-
physiological function
-
both erg24A and erg24B homologs in combination, but not individuall, are essential for the viability of Aspergillus fumigatus. The enzyme plays a critical role in ergosterol biosynthesis and ion homeostasis
-
additional information
TM7SF2 gene expression is controlled by cell sterol levels through the SREBP-2, motifs of TM7SF2 promoter responsible for activation by SREBP-2 are the SRE motif, and both the inverted CCAAT-box and GC-box2, which are essential for full promoter activation by SREBP-2, overview. Binding of SREBP-2 to SRE produces approximately 26fold promoter activation, whereas mutation of the SRE motif causes a dramatic decrease of transactivation by SREBP-2
additional information
the enzyme contains ten transmembrane segments (TM1-10). Its catalytic domain comprises the carboxy-terminal half (containing TM6-10) and envelops two interconnected pockets, one of which faces the cytoplasm and houses NADPH, while the other one is accessible from the lipid bilayer. The reducing end of NADPH meets the sterol substrate at the juncture of the two pockets
additional information
-
the enzyme contains ten transmembrane segments (TM1-10). Its catalytic domain comprises the carboxy-terminal half (containing TM6-10) and envelops two interconnected pockets, one of which faces the cytoplasm and houses NADPH, while the other one is accessible from the lipid bilayer. The reducing end of NADPH meets the sterol substrate at the juncture of the two pockets
additional information
-
the enzyme contains ten transmembrane segments (TM1-10). Its catalytic domain comprises the carboxy-terminal half (containing TM6-10) and envelops two interconnected pockets, one of which faces the cytoplasm and houses NADPH, while the other one is accessible from the lipid bilayer. The reducing end of NADPH meets the sterol substrate at the juncture of the two pockets
-
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D363A
site-directed mutagenesis, inactive mutant
I151M
site-directed mutagenesis, inactive mutant
K406A
site-directed mutagenesis, inactive mutant
L304M
site-directed mutagenesis, inactive mutant
N359A
site-directed mutagenesis, inactive mutant
R395A
site-directed mutagenesis, inactive mutant
W352A
site-directed mutagenesis, inactive mutant
Y241F
site-directed mutagenesis, inactive mutant
Y360A
site-directed mutagenesis, inactive mutant
Y407A
site-directed mutagenesis, inactive mutant
Y414A
site-directed mutagenesis, inactive mutant
D363A
-
site-directed mutagenesis, inactive mutant
-
N359A
-
site-directed mutagenesis, inactive mutant
-
W352A
-
site-directed mutagenesis, inactive mutant
-
Y407A
-
site-directed mutagenesis, inactive mutant
-
Y414A
-
site-directed mutagenesis, inactive mutant
-
D207N
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
D227N
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
D276N
-
site-directed mutagenesis, mutant cannot complement enzyme-deficient erg-3 mutant strain mat a
D289E
-
site-directed mutagenesis, mutant cannot complement enzyme-deficient erg-3 mutant strain mat a
D289N
-
site-directed mutagenesis, mutant cannot complement enzyme-deficient erg-3 mutant strain mat a
D293N
-
site-directed mutagenesis, mutant cannot complement enzyme-deficient erg-3 mutant strain mat a
D394N
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
D462N
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
D463N
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
E22Q
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
E233D
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
E233Q
-
site-directed mutagenesis, mutant cannot complement enzyme-deficient erg-3 mutant strain mat a
E475Q
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
G225C
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
H388A
-
site-directed mutagenesis, mutant cannot complement enzyme-deficient erg-3 mutant strain mat a
H457A
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
K229A
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
K351A
-
site-directed mutagenesis, mutant cannot complement enzyme-deficient erg-3 mutant strain mat a
K351R
-
site-directed mutagenesis, mutant cannot complement enzyme-deficient erg-3 mutant strain mat a
K469A
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
R212A
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
R217A
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
R235A
-
site-directed mutagenesis, mutant cannot complement enzyme-deficient erg-3 mutant strain mat a
R235K
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
R345A
-
site-directed mutagenesis, mutant cannot complement enzyme-deficient erg-3 mutant strain mat a
R355A
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
R458A
-
site-directed mutagenesis, mutant cannot complement enzyme-deficient erg-3 mutant strain mat a
R458K
-
site-directed mutagenesis, mutant cannot complement enzyme-deficient erg-3 mutant strain mat a
R461A
-
site-directed mutagenesis, mutant cannot complement enzyme-deficient erg-3 mutant strain mat a
R461K
-
site-directed mutagenesis, mutant cannot complement enzyme-deficient erg-3 mutant strain mat a
T371A
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
Y124F
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
Y273F
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
Y317F
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
Y391F
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
Y445F
-
site-directed mutagenesis, mutant cannot complement enzyme-deficient erg-3 mutant strain mat a
Y447F
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
Y470F
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
Y477F
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
Y490F
-
site-directed mutagenesis, mutant can complement enzyme-deficient erg-3 mutant strain mat a
additional information
-
mutational blockage of the enzyme leads to accumulation of atypical products from substrate 4alpha-methyl-5alpha-ergosta-8,14,24(28)-trien-3beta-ol in vivo, i.e. 5alpha-cholesta-8,14-dien-3beta-ol, (24R)-5alpha-ergosta-8,14-dien-3beta-ol, and (24R)-5alpha-stigmasta-8,14-dien-3beta-ol
additional information
-
erg24 gene disruption mutant strains, i.e. strain NJ25, NJ50, NJ51, and NJ55, show growth, but altered phenotypes, e.g. increased sensitivity against an allylamine antifungal compound, overview
additional information
enzyme deficiency due to mutations in the lamin B receptor gene causes autosomal recessive HEM/Greenberg skeletal dysplasia
additional information
enzyme deficiency due to mutations in the lamin B receptor gene causes autosomal recessive HEM/Greenberg skeletal dysplasia
additional information
-
enzyme deficiency due to mutations in the lamin B receptor gene causes autosomal recessive HEM/Greenberg skeletal dysplasia
additional information
generation of gene Tm7sf2 knockout mice
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Sterol dependent regulation of human TM7SF2 gene expression: role of the encoded 3beta-hydroxysterol DELTA14-reductase in human cholesterol biosynthesis
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1761
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Saccharomyces cerevisiae
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Fusarium graminearum
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Impaired cell proliferation in regenerating liver of 3beta-hydroxysterol DELTA14-reductase (TM7SF2) knock-out mice
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Mus musculus (Q71KT5)
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Li, X.; Roberti, R.; Blobel, G.
Structure of an integral membrane sterol reductase from Methylomicrobium alcaliphilum
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Methylotuvimicrobium alcaliphilum (G4SW86), Methylotuvimicrobium alcaliphilum, Methylotuvimicrobium alcaliphilum 20Z (G4SW86), Methylotuvimicrobium alcaliphilum 20Z
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Fackel interacts with gibberellic acid signaling and vernalization to mediate flowering in Arabidopsis
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Arabidopsis thaliana (Q9LDR4)
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The sterol C-14 reductase Erg24 is responsible for ergosterol biosynthesis and ion homeostasis in Aspergillus fumigatus
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Abnormal ergosterol biosynthesis activates transcriptional responses to antifungal azoles
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Neurospora crassa (P38670), Neurospora crassa DSM 1257 (P38670)
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Homo sapiens (O76062), Homo sapiens (Q14739), Homo sapiens
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