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Information on EC 1.3.1.94 - polyprenol reductase for references in articles please use BRENDA:EC1.3.1.94
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EC Tree
IUBMB Comments The reaction occurs in the reverse direction with reduction of the terminal double bond next to the alcohol group. Isolated from human fetal brain tissue but present in all eukaryotes. In mammalian cells dolichols are predominantly 18-21 isoprene units in length.
The enzyme appears in viruses and cellular organisms
Synonyms
srd5a3, steroid 5alpha-reductase type 3, dfg10,
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steroid 5alpha-reductase type 3
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steroid 5alpüha-reductase type 3
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SDR5A3
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ditrans,polycis-dolichol + NADP+ = ditrans,polycis-polyprenol + NADPH + H+
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ditrans,polycis-dolichol:NADP+ 2,3-oxidoreductase
The reaction occurs in the reverse direction with reduction of the terminal double bond next to the alcohol group. Isolated from human fetal brain tissue but present in all eukaryotes. In mammalian cells dolichols are predominantly 18-21 isoprene units in length.
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ditrans,polycis-polyprenol + NADPH + H+
ditrans,polycis-dolichol + NADP+
polyprenol + NADPH + H+
dolichol + NADPH
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ditrans,polycis-polyprenol + NADPH + H+
ditrans,polycis-dolichol + NADP+
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ditrans,polycis-polyprenol + NADPH + H+
ditrans,polycis-dolichol + NADP+
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ditrans,polycis-polyprenol + NADPH + H+
ditrans,polycis-dolichol + NADP+
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polyisoprenoids with 13-18 isoprene residues are preferentially reduced
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Cerebellar Ataxia
Next generation sequencing in a family with autosomal recessive Kahrizi syndrome (OMIM 612713) reveals a homozygous frameshift mutation in SRD5A3.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Adult phenotype and further phenotypic variability in SRD5A3-CDG.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Next generation sequencing in a family with autosomal recessive Kahrizi syndrome (OMIM 612713) reveals a homozygous frameshift mutation in SRD5A3.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Phenotypic Expansion of Congenital Disorder of Glycosylation Due to SRD5A3 Null Mutation.
ditrans,polycis-undecaprenyl-diphosphate synthase [(2e,6e)-farnesyl-diphosphate specific] deficiency
SRD5A3-CDG: A patient with a novel mutation.
Intellectual Disability
Next generation sequencing in a family with autosomal recessive Kahrizi syndrome (OMIM 612713) reveals a homozygous frameshift mutation in SRD5A3.
polyprenol reductase deficiency
Life with too much polyprenol: polyprenol reductase deficiency.
polyprenol reductase deficiency
SRD5A3-CDG: A patient with a novel mutation.
Prostatic Neoplasms
Novel 5 alpha-steroid reductase (SRD5A3, type-3) is overexpressed in hormone-refractory prostate cancer.
Prostatic Neoplasms
Role of dutasteride in pre-clinical ETS fusion-positive prostate cancer models.
Retinal Dystrophies
Association of Steroid 5?-Reductase Type 3 Congenital Disorder of Glycosylation With Early-Onset Retinal Dystrophy.
Vision Disorders
Association of Steroid 5?-Reductase Type 3 Congenital Disorder of Glycosylation With Early-Onset Retinal Dystrophy.
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UniProt
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UniProt
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physiological function
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the activities of yeast cis-prenyltransferases Rer2p and Srt1p and polyprenol reductase are not co-regulated and the reductase may be the rate-limiting enzyme in dolichol synthesis if the amount of polyisoprenoids synthesized exceeds a certain level
physiological function
enzyme is necessary for the reduction of the alpha-isoprene unit of polyprenols to form dolichols, required for synthesis of dolichol-linked monosaccharides and the oligosaccharide precursor used for N-glycosylation
physiological function
Srd5a3 mutation is lethal in mouse and results in an activation of the unfolded protein response pathway
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DFG10_YEAST
Saccharomyces cerevisiae (strain ATCC 204508 / S288c)
253
4
30306
Swiss-Prot
PORED_CAEBR
309
6
35465
Swiss-Prot
PORED_CAEEL
309
7
35430
Swiss-Prot
PORED_DANRE
309
5
35708
Swiss-Prot
PORED_DROME
326
3
38034
Swiss-Prot
PORED_HUMAN
318
6
36521
Swiss-Prot
PORED_MESAU
330
6
38164
Swiss-Prot
PORED_MOUSE
330
6
37936
Swiss-Prot
PORED_RAT
330
7
38092
Swiss-Prot
PORED_XENLA
319
4
36591
Swiss-Prot
PORED_XENTR
308
4
35876
Swiss-Prot
POED1_ARATH
320
5
36313
Swiss-Prot
POED1_ORYSI
354
5
39867
Swiss-Prot
POED1_ORYSJ
354
5
39867
Swiss-Prot
POED2_ARATH
343
5
38678
Swiss-Prot
POED2_ORYSI
353
5
39903
Swiss-Prot
POED2_ORYSJ
353
5
39935
Swiss-Prot
PORED_AILME
318
6
36775
Swiss-Prot
A0A1C6XVD5_PLACH
292
4
34464
TrEMBL
A0A2G9G177_9LAMI
336
4
38795
TrEMBL
A0A384KV59_PLAKH
293
5
33973
TrEMBL
A0A2P6PQ63_ROSCH
263
5
30353
TrEMBL
A0A1J1H9R0_PLARL
275
6
32923
TrEMBL
A0A2P6PQ56_ROSCH
71
1
8111
TrEMBL
A0A1J1GQM1_PLAGA
285
6
34413
TrEMBL
A0A1G4H206_PLAVI
294
6
33819
TrEMBL
A0A1D3LLJ1_PLACH
292
4
34454
TrEMBL
A0A2P6PQ70_ROSCH
337
7
38952
TrEMBL
A0A0Y9ZCU2_PLABE
292
5
34674
TrEMBL
A0A396I9G2_MEDTR
345
5
39508
TrEMBL
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expression in Saccharomyces cerevisiae and HEK-293T cells
expression in Saccharomyces cerevisiae and HEK-293T cells
expression in Saccharomyces cerevisiae and HEK-293T cells
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medicine
loss of function mutations of the SRD5A3 gene cause a multisystemic syndrome with eye malformations, cerebellar vermis hypoplasia, and psychomotor delay. Plasma from patients shows increased level of polyprenoids
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Szkopinska, A.; Swiezewska, E.; Rytka, J.
Interplay between the cis-prenyltransferases and polyprenol reductase in the yeast Saccharomyces cerevisiae
Biochimie
88
271-276
2006
Saccharomyces cerevisiae
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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.
SRD5A3 is required for converting polyprenol to dolichol and is mutated in a congenital glycosylation disorder
Cell
142
203-217
2010
Homo sapiens, Homo sapiens (Q9H8P0), Mus musculus (Q9WUP4)
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Sagami, H.; Kurisaki, A.; Ogura, K.
Formation of dolichol from dehydrodolichol is catalyzed by NADPH-dependent reductase localized in microsomes of rat liver
J. Biol. Chem.
268
10109-10113
1993
Rattus norvegicus
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