BRENDA - Enzyme Database show
show all sequences of 1.14.14.25

Cholesterol 24-hydroxylase: an enzyme of cholesterol turnover in the brain

Russell, D.W.; Halford, R.W.; Ramirez, D.M.; Shah, R.; Kotti, T.; Annu. Rev. Biochem. 78, 1017-1040 (2009)

Data extracted from this reference:

Cloned(Commentary)
Commentary
Organism
expressed in 293 cells
Mus musculus
expressed in Escherichia coli
Homo sapiens
Localization
Localization
Commentary
Organism
GeneOntology No.
Textmining
endoplasmic reticulum
-
Mus musculus
5783
-
microsome
-
Bos taurus
-
-
microsome
-
Rattus norvegicus
-
-
Molecular Weight [Da]
Molecular Weight [Da]
Molecular Weight Maximum [Da]
Commentary
Organism
53000
-
immunoblotting
Mus musculus
Organism
Organism
Primary Accession No. (UniProt)
Commentary
Textmining
Bos taurus
-
-
-
Canis lupus familiaris
-
-
-
Cavia porcellus
-
-
-
Equus caballus
-
-
-
Equus sp.
-
zebra
-
Gallus gallus
-
-
-
Homo sapiens
-
-
-
Macaca mulatta
-
-
-
Mus musculus
-
C57BL/6J and 129S6/SvEv mice
-
Ornithorhynchus anatinus
-
-
-
Oryctolagus cuniculus
-
-
-
Pan troglodytes
-
-
-
Rattus norvegicus
-
-
-
Danio rerio
A5WWJ0
-
-
Xenopus laevis
Q7SYY2
-
-
Source Tissue
Source Tissue
Commentary
Organism
Textmining
brain
-
Bos taurus
-
brain
-
Equus caballus
-
brain
fetus, is largely confined to the brain
Homo sapiens
-
brain
is largely confined to the brain, neuron-specific expression
Mus musculus
-
brain
-
Rattus norvegicus
-
hippocampus
high levels
Mus musculus
-
liver
small amounts, level is 100fold lower in the liver than in the brain
Mus musculus
-
additional information
the enzyme is absent from a cortical section from a cholesterol 24-hydroxylase knockout mouse
Mus musculus
-
neuron
large pyramidal cells and their dendrites in cortical layers II, III, V, and VI. Is absent from nonpyramidal cells that compose layer IV
Mus musculus
-
SH-SY5Y cell
contains cholesterol 24-hydroxylase mRNA but does not synthesize 24S-hydroxycholesterol
Homo sapiens
-
testis
small amounts, mRNA does not appear to be translated into protein in the testis
Mus musculus
-
Substrates and Products (Substrate)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
cholesterol + [reduced NADPH-hemoprotein reductase] + H+ + O2
-
701715
Cavia porcellus
(24S)-24-hydroxycholesterol + [oxidized NADPH-hemoprotein reductase] + H2O
-
-
-
?
cholesterol + [reduced NADPH-hemoprotein reductase] + H+ + O2
-
701715
Mus musculus
(24S)-24-hydroxycholesterol + [oxidized NADPH-hemoprotein reductase] + H2O
-
-
-
?
cholesterol + [reduced NADPH-hemoprotein reductase] + H+ + O2
-
701715
Homo sapiens
(24S)-24-hydroxycholesterol + [oxidized NADPH-hemoprotein reductase] + H2O
-
-
-
?
cholesterol + [reduced NADPH-hemoprotein reductase] + H+ + O2
-
701715
Rattus norvegicus
(24S)-24-hydroxycholesterol + [oxidized NADPH-hemoprotein reductase] + H2O
-
-
-
?
cholesterol + [reduced NADPH-hemoprotein reductase] + H+ + O2
-
701715
Bos taurus
(24S)-24-hydroxycholesterol + [oxidized NADPH-hemoprotein reductase] + H2O
-
-
-
?
cholesterol + [reduced NADPH-hemoprotein reductase] + H+ + O2
-
701715
Oryctolagus cuniculus
(24S)-24-hydroxycholesterol + [oxidized NADPH-hemoprotein reductase] + H2O
-
-
-
?
cholesterol + [reduced NADPH-hemoprotein reductase] + H+ + O2
-
701715
Equus caballus
(24S)-24-hydroxycholesterol + [oxidized NADPH-hemoprotein reductase] + H2O
-
-
-
?
Cofactor
Cofactor
Commentary
Organism
Structure
NADPH
-
Bos taurus
NADPH
-
Cavia porcellus
NADPH
-
Equus caballus
NADPH
-
Oryctolagus cuniculus
NADPH
-
Rattus norvegicus
NADPH
-
Mus musculus
NADPH
-
Homo sapiens
Cloned(Commentary) (protein specific)
Commentary
Organism
expressed in Escherichia coli
Homo sapiens
expressed in 293 cells
Mus musculus
Cofactor (protein specific)
Cofactor
Commentary
Organism
Structure
NADPH
-
Bos taurus
NADPH
-
Cavia porcellus
NADPH
-
Equus caballus
NADPH
-
Oryctolagus cuniculus
NADPH
-
Rattus norvegicus
NADPH
-
Mus musculus
NADPH
-
Homo sapiens
Localization (protein specific)
Localization
Commentary
Organism
GeneOntology No.
Textmining
endoplasmic reticulum
-
Mus musculus
5783
-
microsome
-
Bos taurus
-
-
microsome
-
Rattus norvegicus
-
-
Molecular Weight [Da] (protein specific)
Molecular Weight [Da]
Molecular Weight Maximum [Da]
Commentary
Organism
53000
-
immunoblotting
Mus musculus
Source Tissue (protein specific)
Source Tissue
Commentary
Organism
Textmining
brain
-
Bos taurus
-
brain
-
Equus caballus
-
brain
fetus, is largely confined to the brain
Homo sapiens
-
brain
is largely confined to the brain, neuron-specific expression
Mus musculus
-
brain
-
Rattus norvegicus
-
hippocampus
high levels
Mus musculus
-
liver
small amounts, level is 100fold lower in the liver than in the brain
Mus musculus
-
additional information
the enzyme is absent from a cortical section from a cholesterol 24-hydroxylase knockout mouse
Mus musculus
-
neuron
large pyramidal cells and their dendrites in cortical layers II, III, V, and VI. Is absent from nonpyramidal cells that compose layer IV
Mus musculus
-
SH-SY5Y cell
contains cholesterol 24-hydroxylase mRNA but does not synthesize 24S-hydroxycholesterol
Homo sapiens
-
testis
small amounts, mRNA does not appear to be translated into protein in the testis
Mus musculus
-
Substrates and Products (Substrate) (protein specific)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
cholesterol + [reduced NADPH-hemoprotein reductase] + H+ + O2
-
701715
Cavia porcellus
(24S)-24-hydroxycholesterol + [oxidized NADPH-hemoprotein reductase] + H2O
-
-
-
?
cholesterol + [reduced NADPH-hemoprotein reductase] + H+ + O2
-
701715
Mus musculus
(24S)-24-hydroxycholesterol + [oxidized NADPH-hemoprotein reductase] + H2O
-
-
-
?
cholesterol + [reduced NADPH-hemoprotein reductase] + H+ + O2
-
701715
Homo sapiens
(24S)-24-hydroxycholesterol + [oxidized NADPH-hemoprotein reductase] + H2O
-
-
-
?
cholesterol + [reduced NADPH-hemoprotein reductase] + H+ + O2
-
701715
Rattus norvegicus
(24S)-24-hydroxycholesterol + [oxidized NADPH-hemoprotein reductase] + H2O
-
-
-
?
cholesterol + [reduced NADPH-hemoprotein reductase] + H+ + O2
-
701715
Bos taurus
(24S)-24-hydroxycholesterol + [oxidized NADPH-hemoprotein reductase] + H2O
-
-
-
?
cholesterol + [reduced NADPH-hemoprotein reductase] + H+ + O2
-
701715
Oryctolagus cuniculus
(24S)-24-hydroxycholesterol + [oxidized NADPH-hemoprotein reductase] + H2O
-
-
-
?
cholesterol + [reduced NADPH-hemoprotein reductase] + H+ + O2
-
701715
Equus caballus
(24S)-24-hydroxycholesterol + [oxidized NADPH-hemoprotein reductase] + H2O
-
-
-
?
Expression
Organism
Commentary
Expression
Homo sapiens
gradual increase in mRNA and protein in postnatal brain
up
Mus musculus
expression of the gene in the mouse embryo as early as day 11.5 and gradual increase in mRNA and protein in postnatal brain. Over a 14-day culture period, during which the neurons extend processes and form increasing numbers of synapses but do not divide, there is a gradual increase in cholesterol 24-hydroxylase mRNA and protein
up
General Information
General Information
Commentary
Organism
malfunction
disruption of the cholesterol 24-hydroxylase gene causes an 50% decrease in cholesterol turnover, which is compensated for by an equal decrease in the rate of de novo cholesterol synthesis. Mice lacking cholesterol 24-hydroxylase show that whole-body fatty acid and cholesterol metabolism are normal. Profound learning disabilities in cholesterol 24-hydroxylase-deficient mice are often caused by gross anatomical defects in the subregions of the brain implicated in the behavior. A minimal concentration of 0.2 mM geranylgeraniol is required to restore LTP in hippocampal slices from knockout mice
Mus musculus
physiological function
polymorphisms in the human gene are linked to neurodegenerative disorders, such as Alzheimer's disease
Homo sapiens
physiological function
the capacity to turn over cholesterol via the cholesterol 24-hydroxylase pathway may be acquired early during development of the central nervous system. The enzyme reduces the excretion of sterols from the brain but does not abolish this removal process
Mus musculus
General Information (protein specific)
General Information
Commentary
Organism
malfunction
disruption of the cholesterol 24-hydroxylase gene causes an 50% decrease in cholesterol turnover, which is compensated for by an equal decrease in the rate of de novo cholesterol synthesis. Mice lacking cholesterol 24-hydroxylase show that whole-body fatty acid and cholesterol metabolism are normal. Profound learning disabilities in cholesterol 24-hydroxylase-deficient mice are often caused by gross anatomical defects in the subregions of the brain implicated in the behavior. A minimal concentration of 0.2 mM geranylgeraniol is required to restore LTP in hippocampal slices from knockout mice
Mus musculus
physiological function
polymorphisms in the human gene are linked to neurodegenerative disorders, such as Alzheimer's disease
Homo sapiens
physiological function
the capacity to turn over cholesterol via the cholesterol 24-hydroxylase pathway may be acquired early during development of the central nervous system. The enzyme reduces the excretion of sterols from the brain but does not abolish this removal process
Mus musculus
Expression (protein specific)
Organism
Commentary
Expression
Homo sapiens
gradual increase in mRNA and protein in postnatal brain
up
Mus musculus
expression of the gene in the mouse embryo as early as day 11.5 and gradual increase in mRNA and protein in postnatal brain. Over a 14-day culture period, during which the neurons extend processes and form increasing numbers of synapses but do not divide, there is a gradual increase in cholesterol 24-hydroxylase mRNA and protein
up
Other publictions for EC 1.14.14.25
No.
1st author
Pub Med
title
organims
journal
volume
pages
year
Activating Compound
Application
Cloned(Commentary)
Crystallization (Commentary)
Engineering
General Stability
Inhibitors
KM Value [mM]
Localization
Metals/Ions
Molecular Weight [Da]
Natural Substrates/ Products (Substrates)
Organic Solvent Stability
Organism
Oxidation Stability
Posttranslational Modification
Purification (Commentary)
Reaction
Renatured (Commentary)
Source Tissue
Specific Activity [micromol/min/mg]
Storage Stability
Substrates and Products (Substrate)
Subunits
Temperature Optimum [°C]
Temperature Range [°C]
Temperature Stability [°C]
Turnover Number [1/s]
pH Optimum
pH Range
pH Stability
Cofactor
Ki Value [mM]
pI Value
IC50 Value
Activating Compound (protein specific)
Application (protein specific)
Cloned(Commentary) (protein specific)
Cofactor (protein specific)
Crystallization (Commentary) (protein specific)
Engineering (protein specific)
General Stability (protein specific)
IC50 Value (protein specific)
Inhibitors (protein specific)
Ki Value [mM] (protein specific)
KM Value [mM] (protein specific)
Localization (protein specific)
Metals/Ions (protein specific)
Molecular Weight [Da] (protein specific)
Natural Substrates/ Products (Substrates) (protein specific)
Organic Solvent Stability (protein specific)
Oxidation Stability (protein specific)
Posttranslational Modification (protein specific)
Purification (Commentary) (protein specific)
Renatured (Commentary) (protein specific)
Source Tissue (protein specific)
Specific Activity [micromol/min/mg] (protein specific)
Storage Stability (protein specific)
Substrates and Products (Substrate) (protein specific)
Subunits (protein specific)
Temperature Optimum [°C] (protein specific)
Temperature Range [°C] (protein specific)
Temperature Stability [°C] (protein specific)
Turnover Number [1/s] (protein specific)
pH Optimum (protein specific)
pH Range (protein specific)
pH Stability (protein specific)
pI Value (protein specific)
Expression
General Information
General Information (protein specific)
Expression (protein specific)
KCat/KM [mM/s]
KCat/KM [mM/s] (protein specific)
735877
Boussicault
CYP46A1, the rate-limiting enz ...
Homo sapiens, Mus musculus, Mus musculus C57BL/6
Brain
139
953-970
2016
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2
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1
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3
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4
4
-
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-
736502
Anderson
Cholesterol hydroxylase CYP46A ...
Homo sapiens
J. Biol. Chem.
291
11876-81186
2016
2
1
1
1
7
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1
1
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1
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1
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7
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1
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1
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2
2
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736072
Uto
Imidazo[1,2-a]pyridines as cho ...
Homo sapiens
Expert Opin. Ther. Pat.
25
373-377
2015
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1
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11
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1
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1
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1
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1
1
-
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735602
Fourgeux
In vivo consequences of choles ...
Rattus norvegicus, Rattus norvegicus Wistar
Biochem. Biophys. Res. Commun.
446
775-781
2014
-
-
1
-
-
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1
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2
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3
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5
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2
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1
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1
1
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1
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2
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5
-
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2
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1
1
1
1
-
-
728617
Maioli
Is it possible to improve memo ...
Homo sapiens
PLoS ONE
8
e68534
2013
-
-
1
-
-
-
-
-
-
-
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1
-
5
-
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4
-
-
1
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1
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1
1
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1
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4
-
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1
-
-
-
-
-
-
-
-
-
-
1
1
-
-
-
712869
Liao
Quantification of cholesterol- ...
Homo sapiens
J. Proteome Res.
10
241-248
2011
-
-
1
-
-
-
-
-
1
-
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2
-
3
-
-
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-
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6
-
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2
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1
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1
1
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1
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2
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8
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2
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-
727298
Fourgeux
24S-hydroxycholesterol and cho ...
Homo sapiens
Chem. Phys. Lipids
164
496-499
2011
-
-
-
-
-
-
-
-
1
-
-
1
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2
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2
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1
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1
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1
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1
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2
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1
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-
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1
1
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-
705008
Shafaati
The antifungal drug voriconazo ...
Homo sapiens, Mus musculus
J. Lipid Res.
51
318-323
2010
-
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1
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2
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4
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1
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2
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2
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2
1
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1
2
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1
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1
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2
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2
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1
1
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705825
Hudry
Adeno-associated Virus gene th ...
Homo sapiens, Mus musculus, Mus musculus APP23
Mol. Ther.
18
44-53
2010
-
-
1
-
1
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7
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3
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3
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3
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3
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2
2
-
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-
711641
Cartagena
24S-hydroxycholesterol effects ...
Homo sapiens
Brain Res.
1319
1-12
2010
-
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1
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4
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1
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1
3
3
1
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712120
Smiljanic
Brain injury induces cholester ...
Rattus norvegicus, Rattus norvegicus Wistar
Histochem. Cell Biol.
134
159-169
2010
-
-
-
-
-
-
-
-
-
-
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-
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167
-
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4
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4
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1
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1
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712816
Tian
Increased expression of choles ...
Homo sapiens
J. Neurochem.
113
978-989
2010
-
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1
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3
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1
1
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695874
Mast
Combined use of mass spectrome ...
Homo sapiens
Arch. Biochem. Biophys.
483
81-89
2009
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1
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1
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2
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1
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1
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1
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1
1
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698342
Fu
Cholesterol 24-hydroxylase (CY ...
Homo sapiens
Int. J. Geriatr. Psychiatry
24
921-926
2009
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1
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1
1
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701715
Russell
Cholesterol 24-hydroxylase: an ...
Bos taurus, Canis lupus familiaris, Cavia porcellus, Danio rerio, Equus caballus, Equus sp., Gallus gallus, Homo sapiens, Macaca mulatta, Mus musculus, Ornithorhynchus anatinus, Oryctolagus cuniculus, Pan troglodytes, Rattus norvegicus, Xenopus laevis
Annu. Rev. Biochem.
78
1017-1040
2009
-
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2
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3
-
1
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17
-
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-
-
11
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7
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CYP46A1 variants influence Alz ...
Homo sapiens
Eur. Psychiatry
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Fourgeux
Primary open angle glaucoma: A ...
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Plasma levels of 24S-hydroxych ...
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Cortical injury increases chol ...
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Mast
Crystal structures of substrat ...
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Mast
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Mast
Broad substrate specificity of ...
Homo sapiens
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Lund
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Lund
cDNA cloning of cholesterol 24 ...
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