BRENDA - Enzyme Database
show all sequences of 1.3.1.B13

SRD5A3 is required for converting polyprenol to dolichol and is mutated in a congenital glycosylation disorder

Cantagrel, V.; Lefeber, D.J.; Ng, B.G.; Guan, Z.; Silhavy, J.L.; Bielas, S.L.; Lehle, L.; Hombauer, H.; Adamowicz, M.; Swiezewska, E.; De Brouwer, A.P.; Bluemel, P.; Sykut-Cegielska, J.; Houliston, S.; Swistun, D.; Ali, B.R.; Dobyns, W.B.; Babovic-Vuksanovic, D.; van Bokhoven, H.; Wevers, R.A.; Raetz, C.; Cell 142, 203-217 (2010)

Data extracted from this reference:

Cloned(Commentary)
Cloned (Commentary)
Organism
expression in HEK293T cells
Homo sapiens
Engineering
Protein Variants
Commentary
Organism
H296G
inactive mutant enzyme, shows no reduction of polyprenol
Homo sapiens
Natural Substrates/ Products (Substrates)
Natural Substrates
Organism
Commentary (Nat. Sub.)
Natural Products
Commentary (Nat. Pro.)
Organism (Nat. Pro.)
Reversibility
ID
a polyprenol + NADPH + H+
Homo sapiens
the enzyme (SRD5A3) is necessary for the reduction of the alpha-isoprene unit of polyprenols to form alpha-reduced dolichols, required for synthesis of dolichol-linked monosaccharides, and the oligosaccharide precursor used for N-glycosylation. The presence of residual dolichol in cells depleted for this enzyme suggests the existence of an unexpected alternative pathway for dolichol de novo biosynthesis
a dolichol + NADP+
-
-
?
Organism
Organism
UniProt
Commentary
Textmining
Homo sapiens
Q9H8P0
-
-
Substrates and Products (Substrate)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
Substrate Product ID
a dolichol + NADP+
reduction of the alpha-isoprene subunit of polyprenol
719256
Homo sapiens
a polyprenol + NADPH + H+
-
-
-
?
a polyprenol + NADPH + H+
the enzyme (SRD5A3) is necessary for the reduction of the alpha-isoprene unit of polyprenols to form alpha-reduced dolichols, required for synthesis of dolichol-linked monosaccharides, and the oligosaccharide precursor used for N-glycosylation. The presence of residual dolichol in cells depleted for this enzyme suggests the existence of an unexpected alternative pathway for dolichol de novo biosynthesis
719256
Homo sapiens
a dolichol + NADP+
-
-
-
?
Synonyms
Synonyms
Commentary
Organism
SRD5A3
-
Homo sapiens
Temperature Optimum [°C]
Temperature Optimum [°C]
Temperature Optimum Maximum [°C]
Commentary
Organism
37
-
assay at
Homo sapiens
pH Optimum
pH Optimum Minimum
pH Optimum Maximum
Commentary
Organism
8
-
assay at
Homo sapiens
Cofactor
Cofactor
Commentary
Organism
Structure
NADPH
-
Homo sapiens
Cloned(Commentary) (protein specific)
Commentary
Organism
expression in HEK293T cells
Homo sapiens
Cofactor (protein specific)
Cofactor
Commentary
Organism
Structure
NADPH
-
Homo sapiens
Engineering (protein specific)
Protein Variants
Commentary
Organism
H296G
inactive mutant enzyme, shows no reduction of polyprenol
Homo sapiens
Natural Substrates/ Products (Substrates) (protein specific)
Natural Substrates
Organism
Commentary (Nat. Sub.)
Natural Products
Commentary (Nat. Pro.)
Organism (Nat. Pro.)
Reversibility
ID
a polyprenol + NADPH + H+
Homo sapiens
the enzyme (SRD5A3) is necessary for the reduction of the alpha-isoprene unit of polyprenols to form alpha-reduced dolichols, required for synthesis of dolichol-linked monosaccharides, and the oligosaccharide precursor used for N-glycosylation. The presence of residual dolichol in cells depleted for this enzyme suggests the existence of an unexpected alternative pathway for dolichol de novo biosynthesis
a dolichol + NADP+
-
-
?
Substrates and Products (Substrate) (protein specific)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
ID
a dolichol + NADP+
reduction of the alpha-isoprene subunit of polyprenol
719256
Homo sapiens
a polyprenol + NADPH + H+
-
-
-
?
a polyprenol + NADPH + H+
the enzyme (SRD5A3) is necessary for the reduction of the alpha-isoprene unit of polyprenols to form alpha-reduced dolichols, required for synthesis of dolichol-linked monosaccharides, and the oligosaccharide precursor used for N-glycosylation. The presence of residual dolichol in cells depleted for this enzyme suggests the existence of an unexpected alternative pathway for dolichol de novo biosynthesis
719256
Homo sapiens
a dolichol + NADP+
-
-
-
?
Temperature Optimum [°C] (protein specific)
Temperature Optimum [°C]
Temperature Optimum Maximum [°C]
Commentary
Organism
37
-
assay at
Homo sapiens
pH Optimum (protein specific)
pH Optimum Minimum
pH Optimum Maximum
Commentary
Organism
8
-
assay at
Homo sapiens
General Information
General Information
Commentary
Organism
malfunction
mutations in the SRD5A3 gene results in mental retardation, ophthalmologic and cerebellar defects of the patients
Homo sapiens
metabolism
the enzyme (SRD5A3) is necessary for the reduction of the alpha-isoprene unit of polyprenols to form alpha-reduced dolichols, required for synthesis of dolichol-linked monosaccharides, and the oligosaccharide precursor used for N-glycosylation. The presence of residual dolichol in cells depleted for this enzyme suggests the existence of an unexpected alternative pathway for dolichol de novo biosynthesis
Homo sapiens
General Information (protein specific)
General Information
Commentary
Organism
malfunction
mutations in the SRD5A3 gene results in mental retardation, ophthalmologic and cerebellar defects of the patients
Homo sapiens
metabolism
the enzyme (SRD5A3) is necessary for the reduction of the alpha-isoprene unit of polyprenols to form alpha-reduced dolichols, required for synthesis of dolichol-linked monosaccharides, and the oligosaccharide precursor used for N-glycosylation. The presence of residual dolichol in cells depleted for this enzyme suggests the existence of an unexpected alternative pathway for dolichol de novo biosynthesis
Homo sapiens
Other publictions for EC 1.3.1.B13
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
Synonyms
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)
743987
Wheeler
SRD5A3-CDG Expanding the phe ...
Homo sapiens
Am. J. Med. Genet. A
170
3165-3171
2016
-
-
-
-
-
-
-
-
-
-
-
1
-
1
-
-
-
-
-
-
-
-
1
-
2
-
-
-
-
-
-
-
1
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
2
2
-
-
-
746028
Jozwiak
Polyprenol reductase2 deficie ...
no activity in Escherichia coli, Arabidopsis thaliana, Arabidopsis thaliana Col-0
Plant Cell
27
3336-3353
2015
-
-
2
-
4
-
-
-
4
-
-
4
-
13
-
-
-
-
-
10
-
-
4
-
6
-
-
-
-
-
-
-
2
-
-
-
-
-
2
2
-
4
-
-
-
-
-
4
-
-
4
-
-
-
-
-
10
-
-
4
-
-
-
-
-
-
-
-
-
-
6
6
-
-
-
719256
Cantagrel
SRD5A3 is required for convert ...
Homo sapiens
Cell
142
203-217
2010
-
-
1
-
1
-
-
-
-
-
-
1
-
1
-
-
-
-
-
-
-
-
2
-
1
1
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1
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1
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-
1
1
-
1
-
-
-
-
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1
-
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-
-
-
-
-
2
-
1
-
-
-
1
-
-
-
-
2
2
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