Any feedback?
Information on EC 2.5.1.87 - ditrans,polycis-polyprenyl diphosphate synthase [(2E,6E)-farnesyl diphosphate specific]
for references in articles please use BRENDA:EC2.5.1.87Please wait a moment until all data is loaded. This message will disappear when all data is loaded.
EC Tree
2.5.1.87 ditrans,polycis-polyprenyl diphosphate synthase [(2E,6E)-farnesyl diphosphate specific]IUBMB Comments
The enzyme is involved in biosynthesis of dolichol (a long-chain polyprenol) with a saturated alpha-isoprene unit, which serves as a glycosyl carrier in protein glycosylation . The yeast Saccharomyces cerevisiae has two different enzymes that catalyse this reaction. Rer2p synthesizes a well-defined family of polyprenols of 13--18 isoprene residues with dominating C(80) (16 isoprene residues) extending to C(120), while Srt1p synthesizes mainly polyprenol with 22 isoprene subunits. Largest Srt1p products reach C(290) . The enzyme from Arabidopsis thaliana catalyses the formation of polyprenyl diphosphates with predominant carbon number C(120) .
Specify your search results
Word Map
- 2.5.1.87
- dolichols
- cis-prenyltransferase
- pigmentosa
- dehydrodolichol
-
jewish
-
nogos
-
myoclonus
-
ashkenazi
- npc2
- polyprenols
The enzyme appears in viruses and cellular organisms
Reaction Schemes
Synonyms
dhdds, nogo-b receptor, rer2p, tk-idsb, srt1p, dedol-pp synthase, slcpt3, cis-ptase, dedolpp synthase, 
more
topPlease wait a moment until the data is sorted. This message will disappear when the data is sorted.
SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
cis-prenyltransferase
cis-PTase
Dedol-PP synthase
dehydrodolichyl diphosphate synthase
NgBR
Nogo-B receptor
RER2
Rer2p
SlCPT3
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate = n diphosphate + ditrans,polycis-polyprenyl diphosphate (n: 10-55)
-
-
-
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
PATHWAY SOURCE
PATHWAYS
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
SYSTEMATIC NAME
IUBMB Comments
(2E,6E)-farnesyl-diphosphate:isopentenyl-diphosphate cistransferase (adding 1055 isopentenyl units)
The enzyme is involved in biosynthesis of dolichol (a long-chain polyprenol) with a saturated alpha-isoprene unit, which serves as a glycosyl carrier in protein glycosylation [1]. The yeast Saccharomyces cerevisiae has two different enzymes that catalyse this reaction. Rer2p synthesizes a well-defined family of polyprenols of 13--18 isoprene residues with dominating C(80) (16 isoprene residues) extending to C(120), while Srt1p synthesizes mainly polyprenol with 22 isoprene subunits. Largest Srt1p products reach C(290) [2]. The enzyme from Arabidopsis thaliana catalyses the formation of polyprenyl diphosphates with predominant carbon number C(120) [4].
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
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
(2E,6E)-farnesyl diphosphate + 13 isopentenyl diphosphate
13 diphosphate + di-trans,poly-cis-hexadecaprenyl diphosphate
(2E,6E)-farnesyl diphosphate + 14 isopentenyl diphosphate
14 diphosphate + di-trans,poly-cis-heptadecaprenyl diphosphate
-
di-trans,poly-cis-hexadecaprenyl diphosphate and di-trans,poly-cis-heptadecaprenyl diphosphate are the main products
-
?
(2E,6E)-farnesyl diphosphate + 19 isopentenyl diphosphate
19 diphosphate + di-trans,poly-cis-docosaprenyl diphosphate
-
dominating polyprenol with 22 isoprene residues, Srt1p products reaching C290 indicate the failure of a strict bacterial-like chain length control
-
?
(2E,6E)-farnesyl diphosphate + 21 isopentenyl diphosphate
21 diphosphate + di-trans,poly-cis tetracosaprenyl diphosphate
(2E,6E)-farnesyl diphosphate + 5 isopentenyl diphosphate
5 diphosphate + di-trans,poly-cis-octaprenyl diphosphate
(2E,6E)-farnesyl diphosphate + 6 isopentenyl diphosphate
6 diphosphate + di-trans,poly-cis-nonaprenyl diphosphate
(2E,6E)-farnesyl diphosphate + isopentenyl diphosphate
diphosphate + di-trans,poly-cis-polyprenyl diphosphate
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + dehydrodolichyl diphosphate
-
-
-
?
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + di-trans,poly-cis-polyprenyl diphosphate
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate (n: 10-55)
dimethylallyl diphosphate + n isopentenyl diphosphate
n diphosphate + di-trans,poly-cis-polyprenyl diphosphate
-
weak activity
-
-
?
geranyl diphosphate + n isopentenyl diphosphate
n diphosphate + di-trans,poly-cis-polyprenyl diphosphate
-
the enzyme preferably utilizes both geranyl diphosphate and farnesyl diphosphate as the starting substrate
Tk-IdsB produces cis-polyprenyl diphosphates ranging from C15 to C90. Above C75: 11.5%. C65C70: 60.7%. C55C60: 17.4%. C45C50: 3.3%. C30C40: 3.9%. Below C25: 3.2%, mainly yields the C60C65 products
-
?
geranylgeranyl diphosphate + 20 isopentenyl diphosphate
20 diphosphate + di-trans,poly-cis-tetracosaprenyl diphosphate
trans,trans-farnesyl diphosphate is a better substrate than geranylgeranyl diphosphate
the enzyme synthesizes polyisoprenes with carbon number higher than 90. The peak activity is observed at the point that corresponds to the polyisoprene with carbon number C120
-
?
(2E,6E)-farnesyl diphosphate + 13 isopentenyl diphosphate
13 diphosphate + di-trans,poly-cis-hexadecaprenyl diphosphate
-
di-trans,poly-cis-hexadecaprenyl diphosphate and di-trans,poly-cis-heptadecaprenyl diphosphate are the main products
-
?
(2E,6E)-farnesyl diphosphate + 13 isopentenyl diphosphate
13 diphosphate + di-trans,poly-cis-hexadecaprenyl diphosphate
Rer2p Z-prenyltransferase synthesizes a well-defined family of polyprenols of 1318 isoprene residues with dominating C80 (16 isoprene residues) extending to C120. Two polyisoprenoid families with dominating C80 and C110 indicating the activity of both yeast Z-prenyltransferases Rer2p and Srt1p, respectively. The family of longer chain dolichols appearing in the stationary growth phase contains dolichols with even more than 50 isoprene units exhibiting monotonically decreasing amounts with increasing chain lengths. The family of shorter chain dolichols is characteristic of the Rer2p products synthesized during the logarithmic growth phase of yeast. It forms a well-defined family of dolichols with 1318 isoprene residues with a certain level of longer dolichols composed of up to 24 isoprene units
-
-
?
(2E,6E)-farnesyl diphosphate + 13 isopentenyl diphosphate
13 diphosphate + di-trans,poly-cis-hexadecaprenyl diphosphate
Rer2p Z-prenyltransferase synthesizes a well-defined family of polyprenols of 1318 isoprene residues with dominating C80 (16 isoprene residues) extending to C120
-
-
?
(2E,6E)-farnesyl diphosphate + 21 isopentenyl diphosphate
21 diphosphate + di-trans,poly-cis tetracosaprenyl diphosphate
trans,trans-farnesyl diphosphate is a better substrate than geranylgeranyl diphosphate. The enzyme catalyzes the formation of polyprenyl diphosphates with predominant carbon number C120. In vitro rubber biosynthesis analysis indicates that the Arabidopsis cis-prenyltransferase itself could not catalyze the formation of high molecular weight polyprenyl diphosphate such as natural rubber
-
-
?
(2E,6E)-farnesyl diphosphate + 21 isopentenyl diphosphate
21 diphosphate + di-trans,poly-cis tetracosaprenyl diphosphate
trans,trans-farnesyl diphosphate is a better substrate than geranylgeranyl diphosphate
the enzyme synthesizes polyisoprenes with carbon number higher than 90. The peak activity is observed at the point that corresponds to the polyisoprene with carbon number C120
-
?
(2E,6E)-farnesyl diphosphate + 5 isopentenyl diphosphate
5 diphosphate + di-trans,poly-cis-octaprenyl diphosphate
-
di-trans,poly-cis-octaprenyl diphosphate and di-trans,poly-cis-nonaprenyl diphosphate are the main products
-
?
(2E,6E)-farnesyl diphosphate + 5 isopentenyl diphosphate
5 diphosphate + di-trans,poly-cis-octaprenyl diphosphate
the polyprenol product of Srt1p is longer in chain length than that of Rer2p and is not sufficiently converted to dolichol and dolichyl phosphate, unlike that of Rer2p
di-trans,poly-cis-octaprenyl diphosphate and di-trans,poly-cis-nonaprenyl diphosphate are the main products
-
?
(2E,6E)-farnesyl diphosphate + 6 isopentenyl diphosphate
6 diphosphate + di-trans,poly-cis-nonaprenyl diphosphate
-
di-trans,poly-cis-octaprenyl diphosphate and di-trans,poly-cis-nonaprenyl diphosphate are the main products
-
?
(2E,6E)-farnesyl diphosphate + 6 isopentenyl diphosphate
6 diphosphate + di-trans,poly-cis-nonaprenyl diphosphate
the polyprenol product of Srt1p is longer in chain length than that of Rer2p and is not sufficiently converted to dolichol and dolichyl phosphate, unlike that of Rer2p
di-trans,poly-cis-octaprenyl diphosphate and di-trans,poly-cis-nonaprenyl diphosphate are the main products
-
?
(2E,6E)-farnesyl diphosphate + isopentenyl diphosphate
diphosphate + di-trans,poly-cis-polyprenyl diphosphate
-
chain length of products shifts downward from C90 and C95 with increasing concentration of the detergents. Maximum activation is observed at the concentration of 2% Triton X-100, 30 mM octyl glucoside, 30 mM 3-[(3-cholamidopropyl)dimethylammonio]-1-propanesulfonate, and 10 mM deoxycholate with the product chain length being C80-C85, C65-C75, C70-C75, and C55-C65, respectively. The activity of Triton X-100 solubilized enzyme is decreased by asolectin, phosphatidylethanolamine, and phosphatidylcholine. The chain lengths of products formed in the presence of these phospholipids are C85 and C90. In the presence of both phosphatidylcholine and Mg2+ the solubilized enzyme is able to produce C90 and C95 dehydrodolichyl diphosphates like native microsomal enzyme
-
-
?
(2E,6E)-farnesyl diphosphate + isopentenyl diphosphate
diphosphate + di-trans,poly-cis-polyprenyl diphosphate
-
-
-
-
?
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + di-trans,poly-cis-polyprenyl diphosphate
-
-
-
?
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + di-trans,poly-cis-polyprenyl diphosphate
-
the enzyme preferably utilizes both geranyl diphosphate and farnesyl diphosphate as the starting substrate
-
-
?
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate
cis-prenyltransferase activity of the Candida albicans Rer2 homologue
n =10-55
-
?
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate
cis-prenyltransferase activity of the Candida albicans Rer2 homologue
n =10-55
-
?
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate
-
n = 10-55
-
?
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate
-
n = 10-55
-
?
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate (n: 10-55)
-
-
-
-
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate (n: 10-55)
-
-
-
-
-
additional information
?
-
the enzyme does not need LEW1 as regulatory protein for its activity
-
-
-
additional information
?
-
the enzyme is responsible for the formation of long-chain dolichols, dolichol-18 to -23, with dolichol-21 dominating
-
-
-
additional information
?
-
dolichol synthesis using isopentenyl diphosphate
-
-
?
additional information
?
-
dolichol synthesis using isopentenyl diphosphate
-
-
?
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
(2E,6E)-farnesyl diphosphate + 13 isopentenyl diphosphate
13 diphosphate + di-trans,poly-cis-hexadecaprenyl diphosphate
(2E,6E)-farnesyl diphosphate + 14 isopentenyl diphosphate
14 diphosphate + di-trans,poly-cis-heptadecaprenyl diphosphate
-
di-trans,poly-cis-hexadecaprenyl diphosphate and di-trans,poly-cis-heptadecaprenyl diphosphate are the main products
-
?
(2E,6E)-farnesyl diphosphate + 19 isopentenyl diphosphate
19 diphosphate + di-trans,poly-cis-docosaprenyl diphosphate
-
dominating polyprenol with 22 isoprene residues, Srt1p products reaching C290 indicate the failure of a strict bacterial-like chain length control
-
?
(2E,6E)-farnesyl diphosphate + 21 isopentenyl diphosphate
21 diphosphate + di-trans,poly-cis tetracosaprenyl diphosphate
trans,trans-farnesyl diphosphate is a better substrate than geranylgeranyl diphosphate. The enzyme catalyzes the formation of polyprenyl diphosphates with predominant carbon number C120. In vitro rubber biosynthesis analysis indicates that the Arabidopsis cis-prenyltransferase itself could not catalyze the formation of high molecular weight polyprenyl diphosphate such as natural rubber
-
-
?
(2E,6E)-farnesyl diphosphate + 5 isopentenyl diphosphate
5 diphosphate + di-trans,poly-cis-octaprenyl diphosphate
the polyprenol product of Srt1p is longer in chain length than that of Rer2p and is not sufficiently converted to dolichol and dolichyl phosphate, unlike that of Rer2p
di-trans,poly-cis-octaprenyl diphosphate and di-trans,poly-cis-nonaprenyl diphosphate are the main products
-
?
(2E,6E)-farnesyl diphosphate + 6 isopentenyl diphosphate
6 diphosphate + di-trans,poly-cis-nonaprenyl diphosphate
the polyprenol product of Srt1p is longer in chain length than that of Rer2p and is not sufficiently converted to dolichol and dolichyl phosphate, unlike that of Rer2p
di-trans,poly-cis-octaprenyl diphosphate and di-trans,poly-cis-nonaprenyl diphosphate are the main products
-
?
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + di-trans,poly-cis-polyprenyl diphosphate
-
the enzyme preferably utilizes both geranyl diphosphate and farnesyl diphosphate as the starting substrate
-
-
?
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate (n: 10-55)
geranyl diphosphate + n isopentenyl diphosphate
n diphosphate + di-trans,poly-cis-polyprenyl diphosphate
-
the enzyme preferably utilizes both geranyl diphosphate and farnesyl diphosphate as the starting substrate
-
-
?
(2E,6E)-farnesyl diphosphate + 13 isopentenyl diphosphate
13 diphosphate + di-trans,poly-cis-hexadecaprenyl diphosphate
-
di-trans,poly-cis-hexadecaprenyl diphosphate and di-trans,poly-cis-heptadecaprenyl diphosphate are the main products
-
?
(2E,6E)-farnesyl diphosphate + 13 isopentenyl diphosphate
13 diphosphate + di-trans,poly-cis-hexadecaprenyl diphosphate
Rer2p Z-prenyltransferase synthesizes a well-defined family of polyprenols of 1318 isoprene residues with dominating C80 (16 isoprene residues) extending to C120. Two polyisoprenoid families with dominating C80 and C110 indicating the activity of both yeast Z-prenyltransferases Rer2p and Srt1p, respectively. The family of longer chain dolichols appearing in the stationary growth phase contains dolichols with even more than 50 isoprene units exhibiting monotonically decreasing amounts with increasing chain lengths. The family of shorter chain dolichols is characteristic of the Rer2p products synthesized during the logarithmic growth phase of yeast. It forms a well-defined family of dolichols with 1318 isoprene residues with a certain level of longer dolichols composed of up to 24 isoprene units
-
-
?
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate
cis-prenyltransferase activity of the Candida albicans Rer2 homologue
n =10-55
-
?
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate
cis-prenyltransferase activity of the Candida albicans Rer2 homologue
n =10-55
-
?
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate
-
n = 10-55
-
?
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate
-
n = 10-55
-
?
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate (n: 10-55)
-
-
-
-
(2E,6E)-farnesyl diphosphate + n isopentenyl diphosphate
n diphosphate + ditrans,polycis-polyprenyl diphosphate (n: 10-55)
-
-
-
-
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
METALS and IONS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
Mg2+
Mg2+
no activity in absence of Mg2+. Rapid increase of enzyme activity with the addition of Mg2+ up 2 mM. Further addition of Mg2+ inhibits the activity
Mg2+
-
required. Native microsomal enzyme exists in association with lipid and Mg2+
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
Mg2+
no activity in absence of Mg2+. Rapid increase of enzyme activity with the addition of Mg2+ up 2 mM. Further addition of Mg2+ inhibits the activity
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
deoxycholate
-
microsomal enzyme and enzyme released from microsomes are maximally activated at 5 mM. In the absence of deoxycholate, the released enzyme is inactive, while the microsomal enzyme has significant activity
Triton X-100
not strictly dependent on TRiton X-100, maximum activity at 0.01% Triton X-100
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Ataxia
A novel variant of dehydrodolichol diphosphate synthase (DHDDS) mutation with adult-onset progressive myoclonus ataxia.
Blindness
Overexpression and Purification of Human Cis-prenyltransferase in Escherichia coli.
Blindness
Purification and characterization of human dehydrodolychil diphosphate synthase (DHDDS) overexpressed in E. coli.
Blindness
Retinal Degeneration Caused by Rod-Specific Dhdds Ablation Occurs without Concomitant Inhibition of Protein N-Glycosylation.
Brain Diseases
De novo DHDDS variants cause a neurodevelopmental and neurodegenerative disorder with myoclonus.
Breast Neoplasms
Nogo-B receptor expression correlates negatively with malignancy grade and ki-67 antigen expression in invasive ductal breast carcinoma.
Breast Neoplasms
Nogo-B receptor increases the resistance of estrogen receptor positive breast cancer to paclitaxel.
Breast Neoplasms
Nogo-B receptor increases the resistance to tamoxifen in estrogen receptor-positive breast cancer cells.
Carcinogenesis
Nogo-B receptor increases the resistance to tamoxifen in estrogen receptor-positive breast cancer cells.
Carcinoma
Expression of Nogo isoforms and Nogo-B receptor (NgBR) in non-small cell lung carcinomas.
Carcinoma, Hepatocellular
A case of fatal Type I congenital disorders of glycosylation (CDG I) associated with low dehydrodolichol diphosphate synthase (DHDDS) activity.
Carcinoma, Hepatocellular
Nogo-B receptor promotes the chemoresistance of human hepatocellular carcinoma via the ubiquitination of p53 protein.
Carcinoma, Hepatocellular
The Nogo-B receptor promotes human hepatocellular carcinoma cell growth via the Akt signal pathway.
Carcinoma, Non-Small-Cell Lung
Nogo-B receptor is required for stabilizing TGF-? type I receptor and promotes the TGF-?1-induced epithelial-to-mesenchymal transition of non-small cell lung cancer.
Carcinoma, Non-Small-Cell Lung
Nogo-B receptor promotes epithelial-mesenchymal transition in non-small cell lung cancer cells through the Ras/ERK/Snail1 pathway.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Mutation of Nogo-B receptor, a subunit of cis-prenyltransferase, causes a congenital disorder of glycosylation.
ditrans,polycis-polyprenyl diphosphate synthase [(2e,6e)-farnesyl diphosphate specific] deficiency
A case of fatal Type I congenital disorders of glycosylation (CDG I) associated with low dehydrodolichol diphosphate synthase (DHDDS) activity.
ditrans,polycis-polyprenyl diphosphate synthase [(2e,6e)-farnesyl diphosphate specific] deficiency
Nogo-B receptor deficiency causes cerebral vasculature defects during embryonic development in mice.
ditrans,polycis-polyprenyl diphosphate synthase [(2e,6e)-farnesyl diphosphate specific] deficiency
Nogo-B receptor deficiency increases liver X receptor alpha nuclear translocation and hepatic lipogenesis through an adenosine monophosphate-activated protein kinase alpha-dependent pathway.
Drug Resistant Epilepsy
Complex Neurological Phenotype Associated with a De Novo DHDDS Mutation in a Boy with Intellectual Disability, Refractory Epilepsy, and Movement Disorder.
Epilepsy, Generalized
A novel variant of dehydrodolichol diphosphate synthase (DHDDS) mutation with adult-onset progressive myoclonus ataxia.
Epilepsy, Generalized
Fifteen-year follow-up of a patient with a DHDDS variant with non-progressive early onset myoclonic tremor and rare generalized epilepsy.
Erectile Dysfunction
Silencing Nogo-B receptor inhibits penile corpus cavernosum vascular smooth muscle cell apoptosis of rats with diabetic erectile dysfunction by down-regulating ICAM-1.
Hyperkinesis
Complex Neurological Phenotype Associated with a De Novo DHDDS Mutation in a Boy with Intellectual Disability, Refractory Epilepsy, and Movement Disorder.
Hypertriglyceridemia
Activation of hepatic Nogo-B receptor expression-A new anti-liver steatosis mechanism of statins.
Intellectual Disability
Complex Neurological Phenotype Associated with a De Novo DHDDS Mutation in a Boy with Intellectual Disability, Refractory Epilepsy, and Movement Disorder.
Lung Neoplasms
Nogo-B receptor is required for stabilizing TGF-? type I receptor and promotes the TGF-?1-induced epithelial-to-mesenchymal transition of non-small cell lung cancer.
Lung Neoplasms
Nogo-B receptor promotes epithelial-mesenchymal transition in non-small cell lung cancer cells through the Ras/ERK/Snail1 pathway.
Melanoma
Prognostic Significance of NOGO-A/B and NOGO-B Receptor Expression in Malignant Melanoma - A Preliminary Study.
Movement Disorders
Complex Neurological Phenotype Associated with a De Novo DHDDS Mutation in a Boy with Intellectual Disability, Refractory Epilepsy, and Movement Disorder.
Myoclonus
A novel variant of dehydrodolichol diphosphate synthase (DHDDS) mutation with adult-onset progressive myoclonus ataxia.
Myoclonus
De novo DHDDS variants cause a neurodevelopmental and neurodegenerative disorder with myoclonus.
Neoplasm Metastasis
Delivery of small interfering RNA against Nogo-B receptor via tumor-acidity responsive nanoparticles for tumor vessel normalization and metastasis suppression.
Neoplasms
Correlation of Expression of CHI3L1 and Nogo-A and their Role in Angiogenesis in Invasive Ductal Breast Carcinoma.
Neoplasms
Delivery of small interfering RNA against Nogo-B receptor via tumor-acidity responsive nanoparticles for tumor vessel normalization and metastasis suppression.
Neoplasms
Nogo-B Receptor Directs Mitochondria-Associated Membranes to Regulate Vascular Smooth Muscle Cell Proliferation.
Neoplasms
Nogo-B receptor expression correlates negatively with malignancy grade and ki-67 antigen expression in invasive ductal breast carcinoma.
Neurodegenerative Diseases
De novo DHDDS variants cause a neurodevelopmental and neurodegenerative disorder with myoclonus.
Retinal Degeneration
Knock-Down DHDDS Expression Induces Photoreceptor Degeneration in Zebrafish.
Retinal Degeneration
Knockdown of Dehydrodolichyl Diphosphate Synthase in the Drosophila Retina Leads to a Unique Pattern of Retinal Degeneration.
Retinal Degeneration
Lack of Overt Retinal Degeneration in a K42E Dhdds Knock-In Mouse Model of RP59.
Retinal Degeneration
Mutation K42E in Dehydrodolichol Diphosphate Synthase (DHDDS) Causes Recessive Retinitis Pigmentosa.
Retinal Degeneration
Retinal Degeneration Caused by Rod-Specific Dhdds Ablation Occurs without Concomitant Inhibition of Protein N-Glycosylation.
Retinal Degeneration
Selective Ablation of Dehydrodolichyl Diphosphate Synthase in Murine Retinal Pigment Epithelium (RPE) Causes RPE Atrophy and Retinal Degeneration.
Retinal Diseases
Knockdown of Dehydrodolichyl Diphosphate Synthase in the Drosophila Retina Leads to a Unique Pattern of Retinal Degeneration.
Retinitis
De novo DHDDS variants cause a neurodevelopmental and neurodegenerative disorder with myoclonus.
Retinitis Pigmentosa
A missense mutation in DHDDS, encoding dehydrodolichyl diphosphate synthase, is associated with autosomal-recessive retinitis pigmentosa in Ashkenazi Jews.
Retinitis Pigmentosa
Aberrant dolichol chain lengths as biomarkers for retinitis pigmentosa caused by impaired dolichol biosynthesis.
Retinitis Pigmentosa
De novo DHDDS variants cause a neurodevelopmental and neurodegenerative disorder with myoclonus.
Retinitis Pigmentosa
Knockdown of Dehydrodolichyl Diphosphate Synthase in the Drosophila Retina Leads to a Unique Pattern of Retinal Degeneration.
Retinitis Pigmentosa
Lack of Overt Retinal Degeneration in a K42E Dhdds Knock-In Mouse Model of RP59.
Retinitis Pigmentosa
Mutation K42E in Dehydrodolichol Diphosphate Synthase (DHDDS) Causes Recessive Retinitis Pigmentosa.
Retinitis Pigmentosa
Overexpression and Purification of Human Cis-prenyltransferase in Escherichia coli.
Retinitis Pigmentosa
Purification and characterization of human dehydrodolychil diphosphate synthase (DHDDS) overexpressed in E. coli.
Retinitis Pigmentosa
Retinal Degeneration Caused by Rod-Specific Dhdds Ablation Occurs without Concomitant Inhibition of Protein N-Glycosylation.
Retinitis Pigmentosa
Two specific mutations are prevalent causes of recessive retinitis pigmentosa in North American patients of Jewish ancestry.
Retinitis Pigmentosa
Whole-exome sequencing links a variant in DHDDS to retinitis pigmentosa.
Status Epilepticus
Phenotype of heterozygous variants of dehydrodolichol diphosphate synthase.
Tremor
Fifteen-year follow-up of a patient with a DHDDS variant with non-progressive early onset myoclonic tremor and rare generalized epilepsy.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
0.0149
(2E,6E)-farnesyl diphosphate
-
pH 8.5, 37°C, enzyme released from microsomes
0.00588
isopentenyl diphosphate
-
65°C, cosubstrate: geranyl diphosphate
0.0132
isopentenyl diphosphate
-
65°C, cosubstrate: farnesyl diphosphate
0.111
isopentenyl diphosphate
-
65°C, cosubstrate: dimethylallyl diphosphate
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
7.4
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
30
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
pI VALUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
expression is anther-specific, gene is differentially expressed during microspore development in the anther. Expression both in the microspore and in the anther wall increases to the maximum level in the anther wall of 35 mm buds, at which stage the tapetum becomes highly active and secretory
brenda
expresses dehydrodolichyl diphosphate synthase mRNA at high levels
brenda
detected at high levels in roots but hardly detected in flowers, leaves, stems and in suspension-cultured cells
brenda
expresses dehydrodolichyl diphosphate synthase mRNA at high levels
brenda
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
Srt1p resides mostly in lipid particles
-
brenda
localized to the continuous endoplasmic reticulum and some dots associated with the endoplasmic reticulum
brenda
peripheral but tightly associated membrane protein, localized to a subregion of the endoplasmic reticulum membrane that is specialized for dolichol synthesis
brenda