UniProtKB - Q5T2R2 (DPS1_HUMAN)
All trans-polyprenyl-diphosphate synthase PDSS1
PDSS1
Functioni
Heterotetrameric enzyme that catalyzes the condensation of farnesyl diphosphate (FPP), which acts as a primer, and isopentenyl diphosphate (IPP) to produce prenyl diphosphates of varying chain lengths and participates in the determination of the side chain of ubiquinone (PubMed:16262699).
Supplies nona and decaprenyl diphosphate, the precursors for the side chain of the isoprenoid quinones ubiquinone-9 (Q9)and ubiquinone-10 (Q10) respectively (PubMed:16262699).
The enzyme adds isopentenyl diphosphate molecules sequentially to farnesyl diphosphate with trans stereochemistry (PubMed:16262699).
1 PublicationCatalytic activityi
- (2E,6E)-farnesyl diphosphate + 7 isopentenyl diphosphate = all-trans-decaprenyl diphosphate + 7 diphosphate1 PublicationEC:2.5.1.911 PublicationThis reaction proceeds in the forward1 Publication direction.
- (2E,6E)-farnesyl diphosphate + 6 isopentenyl diphosphate = all-trans-nonaprenyl diphosphate + 6 diphosphateBy similarityThis reaction proceeds in the forwardBy similarity direction.
Cofactori
: ubiquinone biosynthesis Pathwayi
This protein is involved in the pathway ubiquinone biosynthesis, which is part of Cofactor biosynthesis.View all proteins of this organism that are known to be involved in the pathway ubiquinone biosynthesis and in Cofactor biosynthesis.
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Binding sitei | 134 | Isopentenyl diphosphateBy similarity | 1 | |
Binding sitei | 137 | Isopentenyl diphosphateBy similarity | 1 | |
Binding sitei | 173 | Isopentenyl diphosphateBy similarity | 1 | |
Metal bindingi | 180 | Magnesium 1By similarity | 1 | |
Metal bindingi | 180 | Magnesium 2By similarity | 1 | |
Metal bindingi | 184 | Magnesium 1By similarity | 1 | |
Metal bindingi | 184 | Magnesium 2By similarity | 1 | |
Binding sitei | 189 | Polyprenyl diphosphateBy similarity | 1 | |
Binding sitei | 190 | Isopentenyl diphosphateBy similarity | 1 | |
Binding sitei | 266 | Polyprenyl diphosphateBy similarity | 1 | |
Binding sitei | 267 | Polyprenyl diphosphateBy similarity | 1 | |
Binding sitei | 304 | Polyprenyl diphosphateBy similarity | 1 | |
Binding sitei | 321 | Polyprenyl diphosphateBy similarity | 1 |
GO - Molecular functioni
- all-trans-decaprenyl-diphosphate synthase activity Source: UniProtKB
- metal ion binding Source: UniProtKB-KW
- prenyltransferase activity Source: GO_Central
- protein heterodimerization activity Source: Ensembl
- trans-hexaprenyltranstransferase activity Source: Ensembl
- trans-octaprenyltranstransferase activity Source: Ensembl
GO - Biological processi
- isoprenoid biosynthetic process Source: HGNC-UCL
- ubiquinone biosynthetic process Source: HGNC-UCL
Keywordsi
Molecular function | Transferase |
Biological process | Isoprene biosynthesis, Lipid metabolism, Ubiquinone biosynthesis |
Ligand | Magnesium, Metal-binding |
Enzyme and pathway databases
BRENDAi | 2.5.1.91, 2681 |
PathwayCommonsi | Q5T2R2 |
Reactomei | R-HSA-2142789, Ubiquinol biosynthesis |
SignaLinki | Q5T2R2 |
UniPathwayi | UPA00232 |
Names & Taxonomyi
Protein namesi | Recommended name: All trans-polyprenyl-diphosphate synthase PDSS1CuratedAlternative name(s): All-trans-decaprenyl-diphosphate synthase subunit 1 (EC:2.5.1.911 Publication) Decaprenyl pyrophosphate synthase subunit 1 Decaprenyl-diphosphate synthase subunit 1Imported Solanesyl-diphosphate synthase subunit 1 Trans-prenyltransferase 1 Short name: TPT 1 |
Gene namesi | |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:17759, PDSS1 |
MIMi | 607429, gene |
neXtProti | NX_Q5T2R2 |
VEuPathDBi | HostDB:ENSG00000148459 |
Subcellular locationi
Mitochondrion
- Mitochondrion Curated
Mitochondrion
- mitochondrial matrix Source: Reactome
Other locations
- transferase complex Source: BHF-UCL
Keywords - Cellular componenti
MitochondrionPathology & Biotechi
Involvement in diseasei
Coenzyme Q10 deficiency, primary, 2 (COQ10D2)1 Publication
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_034879 | 308 | D → E in COQ10D2. 1 PublicationCorresponds to variant dbSNP:rs119463988EnsemblClinVar. | 1 |
Keywords - Diseasei
Disease variant, Primary mitochondrial diseaseOrganism-specific databases
DisGeNETi | 23590 |
GeneReviewsi | PDSS1 |
MalaCardsi | PDSS1 |
MIMi | 614651, phenotype |
OpenTargetsi | ENSG00000148459 |
Orphaneti | 254898, Deafness-encephaloneuropathy-obesity-valvulopathy syndrome |
PharmGKBi | PA134982512 |
Miscellaneous databases
Pharosi | Q5T2R2, Tbio |
Genetic variation databases
BioMutai | PDSS1 |
DMDMi | 74744657 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
ChainiPRO_0000123975 | 1 – 415 | All trans-polyprenyl-diphosphate synthase PDSS1Add BLAST | 415 |
Proteomic databases
EPDi | Q5T2R2 |
jPOSTi | Q5T2R2 |
MassIVEi | Q5T2R2 |
MaxQBi | Q5T2R2 |
PaxDbi | Q5T2R2 |
PeptideAtlasi | Q5T2R2 |
PRIDEi | Q5T2R2 |
ProteomicsDBi | 64353 [Q5T2R2-1] 64354 [Q5T2R2-2] 64355 [Q5T2R2-3] |
PTM databases
iPTMneti | Q5T2R2 |
PhosphoSitePlusi | Q5T2R2 |
Expressioni
Gene expression databases
Bgeei | ENSG00000148459, Expressed in mucosa of transverse colon and 195 other tissues |
ExpressionAtlasi | Q5T2R2, baseline and differential |
Genevisiblei | Q5T2R2, HS |
Organism-specific databases
HPAi | ENSG00000148459, Tissue enhanced (intestine, lymphoid tissue) |
Interactioni
Subunit structurei
Heterotetramer composed of 2 PDSS1/DPS1 and 2 PDSS2/DLP1 subunits.
1 PublicationGO - Molecular functioni
- protein heterodimerization activity Source: Ensembl
Protein-protein interaction databases
BioGRIDi | 117125, 22 interactors |
IntActi | Q5T2R2, 8 interactors |
MINTi | Q5T2R2 |
STRINGi | 9606.ENSP00000365388 |
Miscellaneous databases
RNActi | Q5T2R2, protein |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 16 – 35 | DisorderedSequence analysisAdd BLAST | 20 |
Sequence similaritiesi
Phylogenomic databases
eggNOGi | KOG0776, Eukaryota |
GeneTreei | ENSGT00940000153498 |
HOGENOMi | CLU_014015_1_2_1 |
InParanoidi | Q5T2R2 |
OMAi | GKQMRPM |
OrthoDBi | 381154at2759 |
PhylomeDBi | Q5T2R2 |
TreeFami | TF313548 |
Family and domain databases
CDDi | cd00685, Trans_IPPS_HT, 1 hit |
Gene3Di | 1.10.600.10, 1 hit |
InterProi | View protein in InterPro IPR008949, Isoprenoid_synthase_dom_sf IPR000092, Polyprenyl_synt IPR033749, Polyprenyl_synt_CS |
Pfami | View protein in Pfam PF00348, polyprenyl_synt, 1 hit |
SUPFAMi | SSF48576, SSF48576, 1 hit |
PROSITEi | View protein in PROSITE PS00723, POLYPRENYL_SYNTHASE_1, 1 hit PS00444, POLYPRENYL_SYNTHASE_2, 1 hit |
s (3+)i Sequence
Sequence statusi: Complete.
This entry describes 3 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 3 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MASRWWRWRR GCSWKPAARS PGPGSPGRAG PLGPSAAAEV RAQVHRRKGL
60 70 80 90 100
DLSQIPYINL VKHLTSACPN VCRISRFHHT TPDSKTHSGE KYTDPFKLGW
110 120 130 140 150
RDLKGLYEDI RKELLISTSE LKEMSEYYFD GKGKAFRPII VALMARACNI
160 170 180 190 200
HHNNSRHVQA SQRAIALIAE MIHTASLVHD DVIDDASSRR GKHTVNKIWG
210 220 230 240 250
EKKAVLAGDL ILSAASIALA RIGNTTVISI LTQVIEDLVR GEFLQLGSKE
260 270 280 290 300
NENERFAHYL EKTFKKTASL IANSCKAVSV LGCPDPVVHE IAYQYGKNVG
310 320 330 340 350
IAFQLIDDVL DFTSCSDQMG KPTSADLKLG LATGPVLFAC QQFPEMNAMI
360 370 380 390 400
MRRFSLPGDV DRARQYVLQS DGVQQTTYLA QQYCHEAIRE ISKLRPSPER
410
DALIQLSEIV LTRDK
Computationally mapped potential isoform sequencesi
There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basketH0YEE6 | H0YEE6_HUMAN | All trans-polyprenyl-diphosphate sy... | PDSS1 | 158 | Annotation score: |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 30 | G → V in AAH63635 (PubMed:15489334).Curated | 1 | |
Sequence conflicti | 44 | V → A in AAD28559 (PubMed:10972372).Curated | 1 | |
Sequence conflicti | 47 | R → Q in AAD28559 (PubMed:10972372).Curated | 1 | |
Sequence conflicti | 58 | I → F in AAD28559 (PubMed:10972372).Curated | 1 | |
Sequence conflicti | 66 | S → P in AAD28559 (PubMed:10972372).Curated | 1 | |
Sequence conflicti | 72 – 73 | CR → YS in AAD28559 (PubMed:10972372).Curated | 2 | |
Sequence conflicti | 76 | R → Q in AAD28559 (PubMed:10972372).Curated | 1 | |
Sequence conflicti | 83 | D → Y in AAD28559 (PubMed:10972372).Curated | 1 | |
Sequence conflicti | 109 | D → G in AAD28559 (PubMed:10972372).Curated | 1 | |
Sequence conflicti | 114 | L → P in AAD28559 (PubMed:10972372).Curated | 1 | |
Sequence conflicti | 119 | S → T in AAD28559 (PubMed:10972372).Curated | 1 | |
Sequence conflicti | 124 | M → I in AAD28559 (PubMed:10972372).Curated | 1 | |
Sequence conflicti | 131 | G → V in AAD28559 (PubMed:10972372).Curated | 1 | |
Sequence conflicti | 142 | A → V in AAD28559 (PubMed:10972372).Curated | 1 | |
Sequence conflicti | 186 | A → V in BAD97134 (Ref. 3) Curated | 1 | |
Sequence conflicti | 298 | N → D in BAD97134 (Ref. 3) Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_034879 | 308 | D → E in COQ10D2. 1 PublicationCorresponds to variant dbSNP:rs119463988EnsemblClinVar. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_017100 | 1 – 42 | MASRW…AEVRA → MPA in isoform 3. 1 PublicationAdd BLAST | 42 | |
Alternative sequenceiVSP_017101 | 279 – 415 | SVLGC…LTRDK → FPRNECYDHATVQFAWRCRQ SSTVCTTE in isoform 2. 1 PublicationAdd BLAST | 137 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AF118395 mRNA Translation: AAD28559.1 AB210838 mRNA Translation: BAE48216.1 AK223414 mRNA Translation: BAD97134.1 AL390961 Genomic DNA No translation available. BC049211 mRNA Translation: AAH49211.1 BC063635 mRNA Translation: AAH63635.1 |
CCDSi | CCDS31168.1 [Q5T2R2-1] |
RefSeqi | NP_001308907.1, NM_001321978.1 [Q5T2R2-2] NP_055132.2, NM_014317.4 [Q5T2R2-1] |
Genome annotation databases
Ensembli | ENST00000376215.10; ENSP00000365388.5; ENSG00000148459.16 |
GeneIDi | 23590 |
KEGGi | hsa:23590 |
MANE-Selecti | ENST00000376215.10; ENSP00000365388.5; NM_014317.5; NP_055132.2 |
UCSCi | uc001isv.4, human [Q5T2R2-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AF118395 mRNA Translation: AAD28559.1 AB210838 mRNA Translation: BAE48216.1 AK223414 mRNA Translation: BAD97134.1 AL390961 Genomic DNA No translation available. BC049211 mRNA Translation: AAH49211.1 BC063635 mRNA Translation: AAH63635.1 |
CCDSi | CCDS31168.1 [Q5T2R2-1] |
RefSeqi | NP_001308907.1, NM_001321978.1 [Q5T2R2-2] NP_055132.2, NM_014317.4 [Q5T2R2-1] |
3D structure databases
AlphaFoldDBi | Q5T2R2 |
SMRi | Q5T2R2 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 117125, 22 interactors |
IntActi | Q5T2R2, 8 interactors |
MINTi | Q5T2R2 |
STRINGi | 9606.ENSP00000365388 |
PTM databases
iPTMneti | Q5T2R2 |
PhosphoSitePlusi | Q5T2R2 |
Genetic variation databases
BioMutai | PDSS1 |
DMDMi | 74744657 |
Proteomic databases
EPDi | Q5T2R2 |
jPOSTi | Q5T2R2 |
MassIVEi | Q5T2R2 |
MaxQBi | Q5T2R2 |
PaxDbi | Q5T2R2 |
PeptideAtlasi | Q5T2R2 |
PRIDEi | Q5T2R2 |
ProteomicsDBi | 64353 [Q5T2R2-1] 64354 [Q5T2R2-2] 64355 [Q5T2R2-3] |
Protocols and materials databases
Antibodypediai | 25917, 61 antibodies from 17 providers |
DNASUi | 23590 |
Genome annotation databases
Ensembli | ENST00000376215.10; ENSP00000365388.5; ENSG00000148459.16 |
GeneIDi | 23590 |
KEGGi | hsa:23590 |
MANE-Selecti | ENST00000376215.10; ENSP00000365388.5; NM_014317.5; NP_055132.2 |
UCSCi | uc001isv.4, human [Q5T2R2-1] |
Organism-specific databases
CTDi | 23590 |
DisGeNETi | 23590 |
GeneCardsi | PDSS1 |
GeneReviewsi | PDSS1 |
HGNCi | HGNC:17759, PDSS1 |
HPAi | ENSG00000148459, Tissue enhanced (intestine, lymphoid tissue) |
MalaCardsi | PDSS1 |
MIMi | 607429, gene 614651, phenotype |
neXtProti | NX_Q5T2R2 |
OpenTargetsi | ENSG00000148459 |
Orphaneti | 254898, Deafness-encephaloneuropathy-obesity-valvulopathy syndrome |
PharmGKBi | PA134982512 |
VEuPathDBi | HostDB:ENSG00000148459 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG0776, Eukaryota |
GeneTreei | ENSGT00940000153498 |
HOGENOMi | CLU_014015_1_2_1 |
InParanoidi | Q5T2R2 |
OMAi | GKQMRPM |
OrthoDBi | 381154at2759 |
PhylomeDBi | Q5T2R2 |
TreeFami | TF313548 |
Enzyme and pathway databases
UniPathwayi | UPA00232 |
BRENDAi | 2.5.1.91, 2681 |
PathwayCommonsi | Q5T2R2 |
Reactomei | R-HSA-2142789, Ubiquinol biosynthesis |
SignaLinki | Q5T2R2 |
Miscellaneous databases
BioGRID-ORCSi | 23590, 169 hits in 1076 CRISPR screens |
ChiTaRSi | PDSS1, human |
GeneWikii | PDSS1 |
GenomeRNAii | 23590 |
Pharosi | Q5T2R2, Tbio |
PROi | PR:Q5T2R2 |
RNActi | Q5T2R2, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000148459, Expressed in mucosa of transverse colon and 195 other tissues |
ExpressionAtlasi | Q5T2R2, baseline and differential |
Genevisiblei | Q5T2R2, HS |
Family and domain databases
CDDi | cd00685, Trans_IPPS_HT, 1 hit |
Gene3Di | 1.10.600.10, 1 hit |
InterProi | View protein in InterPro IPR008949, Isoprenoid_synthase_dom_sf IPR000092, Polyprenyl_synt IPR033749, Polyprenyl_synt_CS |
Pfami | View protein in Pfam PF00348, polyprenyl_synt, 1 hit |
SUPFAMi | SSF48576, SSF48576, 1 hit |
PROSITEi | View protein in PROSITE PS00723, POLYPRENYL_SYNTHASE_1, 1 hit PS00444, POLYPRENYL_SYNTHASE_2, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | DPS1_HUMAN | |
Accessioni | Q5T2R2Primary (citable) accession number: Q5T2R2 Secondary accession number(s): Q53F75 Q9Y2W5 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | January 24, 2006 |
Last sequence update: | December 21, 2004 | |
Last modified: | May 25, 2022 | |
This is version 151 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. |
Miscellaneousi
Keywords - Technical termi
Reference proteomeDocuments
- Human chromosome 10
Human chromosome 10: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PATHWAY comments
Index of metabolic and biosynthesis pathways - SIMILARITY comments
Index of protein domains and families