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Information on EC 1.14.18.9 - 4alpha-methylsterol monooxygenase and Organism(s) Homo sapiens
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1.14.18.9 4alpha-methylsterol monooxygenaseIUBMB Comments
This enzyme is found in fungi and animals and catalyses a step in the biosynthesis of important sterol molecules such as ergosterol and cholesterol, respectively. The enzyme acts on the 4alpha-methyl group. Subsequent decarboxylation by EC 1.1.1.170, 3beta-hydroxysteroid-4alpha-carboxylate 3-dehydrogenase (decarboxylating), occurs concomitantly with epimerization of the remaining 4beta-methyl into the 4alpha position, thus making it a suitable substrate for a second round of catalysis. cf. EC 1.14.13.246, 4beta-methylsterol monooxygenase; EC 1.14.18.10, plant 4,4-dimethylsterol C-4alpha-methyl-monooxygenase; and EC 1.14.18.11, plant 4alpha-monomethylsterol monooxygenase.
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Word Map
- 1.14.18.9
- ergosterol
- lanosterol
- 4,4-dimethylzymosterol
- erg27p
-
psoriasiform
-
smith-lemli-opitz
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meiosis-activating
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methylsterols
- medicine
- agriculture
The taxonomic range for the selected organisms is: Homo sapiens
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria
Reaction Schemes
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Synonyms
erg25, sc4mol, methyl sterol oxidase, erg25p, 4-methyl sterol oxidase, c-4 sterol methyl oxidase, c-4 methyl sterol oxidase, sterol c4 methyl oxidase, sterol-c4-methyl oxidase, 4,4-dimethyl-zymosterol 4alpha-methyl-oxidase, 
more
topSYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
4-methylsterol oxidase
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methylsterol hydroxylase
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methylsterol monooxygenase
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sterol-C4-methyl oxidase
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
oxidation
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redox reaction
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reduction
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PATHWAY SOURCE
PATHWAYS
BRENDA
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-, -, -, -
SYSTEMATIC NAME
IUBMB Comments
4,4-dimethyl-5alpha-cholest-7-en-3beta-ol,ferrocytochrome-b5:oxygen oxidoreductase (C4alpha-methyl-hydroxylating)
This enzyme is found in fungi and animals and catalyses a step in the biosynthesis of important sterol molecules such as ergosterol and cholesterol, respectively. The enzyme acts on the 4alpha-methyl group. Subsequent decarboxylation by EC 1.1.1.170, 3beta-hydroxysteroid-4alpha-carboxylate 3-dehydrogenase (decarboxylating), occurs concomitantly with epimerization of the remaining 4beta-methyl into the 4alpha position, thus making it a suitable substrate for a second round of catalysis. cf. EC 1.14.13.246, 4beta-methylsterol monooxygenase; EC 1.14.18.10, plant 4,4-dimethylsterol C-4alpha-methyl-monooxygenase; and EC 1.14.18.11, plant 4alpha-monomethylsterol monooxygenase.
CAS REGISTRY NUMBER
COMMENTARY
37256-80-7
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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
4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol + 3 reduced acceptor + 3 O2
(3beta,4alpha)-3-hydroxy-4-methylcholesta-8,14-24-triene-4-carboxylate + 3 acceptor + 3 H2O
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additional information
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the enzyme is involved in cholesterol biosynthesis, (24S)-hydroxycholesterol downregulates cholesterol synthesis in neurons, possibly, in a posttranscriptional manner through SREBP-2, overview
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?
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
additional information
?
-
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the enzyme is involved in cholesterol biosynthesis, (24S)-hydroxycholesterol downregulates cholesterol synthesis in neurons, possibly, in a posttranscriptional manner through SREBP-2, overview
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-
?
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
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(24S)-hydroxycholesterol downregulates the expression of several members of the cholesterol/isoprenoid synthesis pathways including the methylsterol monooxygenase, overview
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
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inhibition of sterol C4methyl oxidase induces activation of the cell cycle
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
malfunction
physiological function
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the enzyme regulates epidermal growth factor receptor signaling and vesicular trafficking
malfunction
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deficiency of sterol C4 methyl oxidase, is associated with microcephaly, congenital cataracts, and growth delay in infancy
malfunction
mutations in methyl sterol oxidase cause psoriasiform dermatitis, arthralgias, congenital cataracts, microcephaly, and developmental delay. Enzyme deficiency also substantially alters immunocyte phenotype and in vitro function
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable).
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable). MSMO1_HUMAN
293
3
35216
Swiss-Prot
other Location (Reliability: 4)
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SUBUNIT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
POSTTRANSLATIONAL MODIFICATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
H173Q
Y244C
H173Q
the mutation is associated with sterol-C4-methyl oxidase deficiency
Y244C
the mutation is associated with sterol-C4-methyl oxidase deficiency
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Wang, Y.; Muneton, S.; Sjoevall, J.; Jovanovic, J.N.; Griffiths, W.J.
The effect of 24S-hydroxycholesterol on cholesterol homeostasis in neurons: quantitative changes to the cortical neuron proteome
J. Proteome Res.
7
1606-1614
2008
Homo sapiens
He, M.; Smith, L.D.; Chang, R.; Li, X.; Vockley, J.
The role of sterol-C4-methyl oxidase in epidermal biology
Biochim. Biophys. Acta
1841
331-335
2013
Homo sapiens
He, M.; Kratz, L.E.; Michel, J.J.; Vallejo, A.N.; Ferris, L.; Kelley, R.I.; Hoover, J.J.; Jukic, D.; Gibson, K.M.; Wolfe, L.A.; Ramachandran, D.; Zwick, M.E.; Vockley, J.
Mutations in the human SC4MOL gene encoding a methyl sterol oxidase cause psoriasiform dermatitis, microcephaly, and developmental delay
J. Clin. Invest.
121
976-984
2011
Homo sapiens (Q15800), Homo sapiens
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