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Information on EC 1.3.1.21 - 7-dehydrocholesterol reductase
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EC Tree
1.3.1.21 7-dehydrocholesterol reductaseIUBMB Comments
The enzyme is part of the cholesterol biosynthesis pathway.
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Word Map
- 1.3.1.21
-
smith-lemli-opitz
- malformation
- cyp2r1
- anomaly
-
25-hydroxyvitamin
-
syndactyly
-
d-related
- desmosterol
- 8-dehydrocholesterol
- dhcr24
-
genitalia
- oxysterols
- holoprosencephaly
- rsh
- ecdysteroids
-
cholesterogenic
- medicine
- diagnostics
-
25-hydroxylase
- trazodone
- aripiprazole
- ebp
- hmgcs1
The enzyme appears in viruses and cellular organisms
Synonyms
dhcr7, 7-dehydrocholesterol reductase, neverland, daf-36, sterol delta7-reductase, 3beta-hydroxysterol delta7-reductase, 7-dhc reductase, 7-dhcr, 3beta-hydroxysterol-delta7-reductase, 7-dehydrocholesterol delta7-reductase, 
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
3beta-hydroxysterol DELTA7-reductase
7-DHC reductase
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-
-
-
Dhcr7
Dwarf5 protein
-
-
-
-
reductase, 7-dehydrocholesterol
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-
-
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Sterol delta-7-reductase
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-
-
-
sterol DELTA7 reductase
sterol DELTA7-reductase
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
cholesterol + NADP+ = cholesta-5,7-dien-3beta-ol + NADPH + H+
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-
-
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cholesterol + NADP+ = cholesta-5,7-dien-3beta-ol + NADPH + H+
cholesta-5,7-dien-3beta-ol is 7-dehydrocholesterol
-
-
-
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
oxidation
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-
-
-
redox reaction
-
-
-
-
reduction
-
-
-
-
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PATHWAY SOURCE
PATHWAYS
BRENDA
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SYSTEMATIC NAME
IUBMB Comments
cholesterol:NADP+ DELTA7-oxidoreductase
The enzyme is part of the cholesterol biosynthesis pathway.
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CAS REGISTRY NUMBER
COMMENTARY
9080-21-1
-
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
20S-hydroxy-7-dehydrocholesterol + NADPH + H+
20S-hdroxycholesterol + NADP+
-
-
-
?
27-hydroxydehydrocholesterol + NADPH + H+
27-hydroxycholesterol + NADP+
-
-
-
?
7-dehydrositosterol + NADPH
sitosterol + NADP+
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25% of that with 7-dehydrocholesterol
-
?
cholesta-5,7-dien-3alpha-ol + NADPH
epicholesterol + NADP+
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i.e. 7-dehydroepicholesterol, 25% activity of that with 7-dehydrocholesterol
-
-
?
cholesterol + NADP+
cholesta-5,7-dien-3-beta-ol + NADPH + H+
-
-
-
?
ergosta-5,7-diene-3beta-ol + NADPH
ergosta-5-eneol + NADP+
-
-
?
7-dehydrodesmosterol + NADPH
desmosterol + NADP+
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terminal step in desmosterol de novo biosynthesis, pathway and physiological role, overview
-
-
?
7-dehydrodesmosterol + NADPH
desmosterol + NADP+
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desmosterol is cholesta-5,24-dienol
-
?
7-dehydrodesmosterol + NADPH
desmosterol + NADP+
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desmosterol is cholesta-5,24-dienol
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
highly regiospecific for DELTA7-bond reduction, but wide substrate specificity
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
-
defect in cholesterol biosynthesis cause multiple congenital, developmental and morphogenic anomalies, e.g. the Smith-Lemli-Opitz syndrome due to a gene mutation, overview
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
-
defect in cholesterol biosynthesis cause multiple congenital, developmental and morphogenic anomalies, e.g. the Smith-Lemli-Opitz syndrome due to a gene mutation, overview
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
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cholesterol biosynthesis
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
-
cholesterol biosynthesis
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
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cholesterol formation outside myelin sheath
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
-
cholesterol biosynthesis
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
-
cholesterol biosynthesis
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
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cholesterol biosynthesis
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
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reversibility of the reaction in vivo could not be proven
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
-
wide substrate spectrum
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
wide substrate spectrum
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
-
wide substrate spectrum
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
enzyme catalyzes the terminal step in cholesterol biosynthesis by reducing 7-dehydrocholesterol
-
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
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final step in cholesterol biosynthesis
-
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
last step in cholesterol biosynthesis
-
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
-
terminal step in cholesterol de novo biosynthesis, pathway and physiological role, overview
-
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
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terminal step in cholesterol de novo biosynthesis, physiological role, overview
-
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
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i.e. 7-dehydrocholesterol
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-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
i.e. 7-dehydrocholesterol
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-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
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i.e. 7-dehydrocholesterol
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-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
-
-
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
enzyme catalyzes the terminal step in cholesterol biosynthesis by reducing 7-dehydrocholesterol
-
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
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i.e. 7-dehydrocholesterol
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-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
i.e. 7-dehydrocholesterol
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-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
-
isotope effects
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
enzyme catalyzes the terminal step in cholesterol biosynthesis by reducing 7-dehydrocholesterol
-
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
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i.e. 7-dehydrocholesterol
-
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
i.e. 7-dehydrocholesterol
-
-
?
cholesterol + NADP+
cholesta-5,7-dien-3beta-ol + NADPH + H+
-
-
-
-
?
cholesterol + O2 + NAD(P)H + H+
cholesta-5,7-dien-3beta-ol + NAD(P)+ + 2 H2O
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-
-
?
cholesterol + O2 + NAD(P)H + H+
cholesta-5,7-dien-3beta-ol + NAD(P)+ + 2 H2O
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-
-
?
cholesterol + O2 + NAD(P)H + H+
cholesta-5,7-dien-3beta-ol + NAD(P)+ + 2 H2O
first committed step in biosynthesis of Caenorhabditis elegans bile acid-like steroids called dafachronic acids
-
-
?
ergosta-5,7,22-trien-3beta-ol + NADPH
ergosta-5,22-diene-3beta-ol + NADP+
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i.e. ergosterol
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?
ergosta-5,7,22-trien-3beta-ol + NADPH
ergosta-5,22-diene-3beta-ol + NADP+
i.e. ergosterol
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?
ergosta-5,7,22-trien-3beta-ol + NADPH
ergosta-5,22-diene-3beta-ol + NADP+
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i.e. ergosterol
i.e. brassicasterol
?
ergosta-5,7,22-trien-3beta-ol + NADPH
ergosta-5,22-diene-3beta-ol + NADP+
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i.e. ergosterol
i.e. brassicasterol
?
ergosta-5,7,22-trien-3beta-ol + NADPH
ergosta-5,22-diene-3beta-ol + NADP+
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25% activity of that with 7-dehydrocholesterol
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?
ergosta-5,7,22-trien-3beta-ol + NADPH
ergosta-5,22-diene-3beta-ol + NADP+
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i.e. ergosterol
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-
?
ergosta-5,7,22-trien-3beta-ol + NADPH
ergosta-5,22-diene-3beta-ol + NADP+
-
i.e. ergosterol
-
?
ergosta-5,7,22-trien-3beta-ol + NADPH
ergosta-5,22-diene-3beta-ol + NADP+
-
i.e. ergosterol
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?
ergosta-5,7,22-trien-3beta-ol + NADPH
ergosta-5,22-diene-3beta-ol + NADP+
-
enzyme activity diet-inducible: 2.6fold by 0.5% ergosterol and 5fold by 5.0% cholestyramine and 0.1% lovastatin
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-
?
ergosta-5,7-diene-3beta-ol + NADPH
ergosta-5-ene-3beta-ol + NADP+
-
-
-
?
ergosta-5,7-diene-3beta-ol + NADPH
ergosta-5-ene-3beta-ol + NADP+
also ergosta-5,24(28)-, ergosta-5,22,24(28)-eneols and cholesta-5,24- or cholesta-5,22,24-eneols can be formed in vivo in the mutants of Saccharomyces cerevisiae
-
?
additional information
?
-
-
a mutational enzyme defect causes the Smith-Lemli-Opitz syndrome, a severe developmental disorder associated with multiple congenital anomalies, with low cholesterol and high precurosor 7-hydrocholesterol contents in plasma and tissues, clinical symptoms, overview
-
-
?
additional information
?
-
-
over 92% reduced enzyme activity, due to autosomal recessive mutational disorder, leads to the Smith-Lemli-Opitz syndrome in multiple cell types
-
-
?
additional information
?
-
reduced enzyme activity due to mutation leads to holoprosencephaly in vivo, phenotype
-
-
?
additional information
?
-
-
reduced enzyme activity due to mutation leads to holoprosencephaly in vivo, phenotype
-
-
?
additional information
?
-
-
several enzyme mutational defects cause the Smith-Lemli-Opitz syndrome, a severe developmental disorder associated with multiple congenital malformations and mental retardation, or desmosterolosis and lathosterolosis, rare autosomal recessive disorders, defect cholesterol synthesis, clinical symptoms, overview
-
-
?
additional information
?
-
enzyme is regulated through tissue-specific transcription and differential alternative splicing
-
-
?
additional information
?
-
-
enzyme is regulated through tissue-specific transcription and differential alternative splicing
-
-
?
additional information
?
-
-
enzyme is essential for steroidogenesis, enzyme is not regulated by the luteinizing hormone LH
-
-
?
additional information
?
-
enzyme is regulated through tissue-specific transcription and differential alternative splicing, enzyme is induced when sterols are depleted both in vivo and in vitro
-
-
?
additional information
?
-
enzyme is regulated through tissue-specific transcription and differential alternative splicing, enzyme is induced when sterols are depleted both in vivo and in vitro
-
-
?
additional information
?
-
no substrates: 8-dehydrocholesterol or 20S-hydroxylumisterol
-
-
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
cholesterol + O2 + NAD(P)H + H+
cholesta-5,7-dien-3beta-ol + NAD(P)+ + 2 H2O
first committed step in biosynthesis of Caenorhabditis elegans bile acid-like steroids called dafachronic acids
-
-
?
7-dehydrodesmosterol + NADPH
desmosterol + NADP+
-
terminal step in desmosterol de novo biosynthesis, pathway and physiological role, overview
-
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
highly regiospecific for DELTA7-bond reduction, but wide substrate specificity
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
-
defect in cholesterol biosynthesis cause multiple congenital, developmental and morphogenic anomalies, e.g. the Smith-Lemli-Opitz syndrome due to a gene mutation, overview
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
-
defect in cholesterol biosynthesis cause multiple congenital, developmental and morphogenic anomalies, e.g. the Smith-Lemli-Opitz syndrome due to a gene mutation, overview
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
-
cholesterol biosynthesis
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
-
cholesterol biosynthesis
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
-
cholesterol formation outside myelin sheath
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
-
cholesterol biosynthesis
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
-
cholesterol biosynthesis
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
-
cholesterol biosynthesis
-
?
cholesta-5,7-dien-3-beta-ol + NADPH
cholesterol + NADP+
-
reversibility of the reaction in vivo could not be proven
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
enzyme catalyzes the terminal step in cholesterol biosynthesis by reducing 7-dehydrocholesterol
-
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
-
final step in cholesterol biosynthesis
-
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
last step in cholesterol biosynthesis
-
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
-
terminal step in cholesterol de novo biosynthesis, pathway and physiological role, overview
-
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
-
terminal step in cholesterol de novo biosynthesis, physiological role, overview
-
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
enzyme catalyzes the terminal step in cholesterol biosynthesis by reducing 7-dehydrocholesterol
-
-
?
cholesta-5,7-dien-3beta-ol + NADPH
cholesterol + NADP+
enzyme catalyzes the terminal step in cholesterol biosynthesis by reducing 7-dehydrocholesterol
-
-
?
cholesterol + NADP+
cholesta-5,7-dien-3beta-ol + NADPH + H+
-
-
-
-
?
ergosta-5,7-diene-3beta-ol + NADPH
ergosta-5-ene-3beta-ol + NADP+
-
-
-
?
ergosta-5,7-diene-3beta-ol + NADPH
ergosta-5-ene-3beta-ol + NADP+
also ergosta-5,24(28)-, ergosta-5,22,24(28)-eneols and cholesta-5,24- or cholesta-5,22,24-eneols can be formed in vivo in the mutants of Saccharomyces cerevisiae
-
?
additional information
?
-
-
a mutational enzyme defect causes the Smith-Lemli-Opitz syndrome, a severe developmental disorder associated with multiple congenital anomalies, with low cholesterol and high precurosor 7-hydrocholesterol contents in plasma and tissues, clinical symptoms, overview
-
-
?
additional information
?
-
-
over 92% reduced enzyme activity, due to autosomal recessive mutational disorder, leads to the Smith-Lemli-Opitz syndrome in multiple cell types
-
-
?
additional information
?
-
reduced enzyme activity due to mutation leads to holoprosencephaly in vivo, phenotype
-
-
?
additional information
?
-
-
reduced enzyme activity due to mutation leads to holoprosencephaly in vivo, phenotype
-
-
?
additional information
?
-
-
several enzyme mutational defects cause the Smith-Lemli-Opitz syndrome, a severe developmental disorder associated with multiple congenital malformations and mental retardation, or desmosterolosis and lathosterolosis, rare autosomal recessive disorders, defect cholesterol synthesis, clinical symptoms, overview
-
-
?
additional information
?
-
enzyme is regulated through tissue-specific transcription and differential alternative splicing
-
-
?
additional information
?
-
-
enzyme is regulated through tissue-specific transcription and differential alternative splicing
-
-
?
additional information
?
-
-
enzyme is essential for steroidogenesis, enzyme is not regulated by the luteinizing hormone LH
-
-
?
additional information
?
-
enzyme is regulated through tissue-specific transcription and differential alternative splicing, enzyme is induced when sterols are depleted both in vivo and in vitro
-
-
?
additional information
?
-
enzyme is regulated through tissue-specific transcription and differential alternative splicing, enzyme is induced when sterols are depleted both in vivo and in vitro
-
-
?