Gene/Proteome Database (LMPD)
LMPD ID
LMP005824
Gene ID
Species
Homo sapiens (Human)
Gene Name
prenyl (decaprenyl) diphosphate synthase, subunit 1
Gene Symbol
Synonyms
COQ1; COQ10D2; DPS; SPS; TPRT; TPT; TPT 1; hDPS1
Alternate Names
decaprenyl-diphosphate synthase subunit 1; coenzyme Q1 homolog; trans-prenyltransferase 1; trans-prenyltransferase (TPT); polyprenyl pyrophosphate synthetase; decaprenyl pyrophosphate synthase subunit 1; subunit 1 of decaprenyl diphosphate synthase; all-trans-decaprenyl-diphosphate synthase subunit 1
Chromosome
10
Map Location
10p12.1
EC Number
2.5.1.91
Summary
The protein encoded by this gene is an enzyme that elongates the prenyl side-chain of coenzyme Q, or ubiquinone, one of the key elements in the respiratory chain. The gene product catalyzes the formation of all trans-polyprenyl pyrophosphates from isopentyl diphosphate in the assembly of polyisoprenoid side chains, the first step in coenzyme Q biosynthesis. The protein may be peripherally associated with the inner mitochondrial membrane, though no transit peptide has been definitively identified to date. Defects in this gene are a cause of coenzyme Q10 deficiency. [provided by RefSeq, Jul 2008]
Orthologs
Proteins
| decaprenyl-diphosphate synthase subunit 1 | |
|---|---|
| Refseq ID | NP_055132 |
| Protein GI | 50659086 |
| UniProt ID | Q5T2R2 |
| mRNA ID | NM_014317 |
| Length | 415 |
| RefSeq Status | REVIEWED |
| MASRWWRWRRGCSWKPAARSPGPGSPGRAGPLGPSAAAEVRAQVHRRKGLDLSQIPYINLVKHLTSACPNVCRISRFHHTTPDSKTHSGEKYTDPFKLGWRDLKGLYEDIRKELLISTSELKEMSEYYFDGKGKAFRPIIVALMARACNIHHNNSRHVQASQRAIALIAEMIHTASLVHDDVIDDASSRRGKHTVNKIWGEKKAVLAGDLILSAASIALARIGNTTVISILTQVIEDLVRGEFLQLGSKENENERFAHYLEKTFKKTASLIANSCKAVSVLGCPDPVVHEIAYQYGKNVGIAFQLIDDVLDFTSCSDQMGKPTSADLKLGLATGPVLFACQQFPEMNAMIMRRFSLPGDVDRARQYVLQSDGVQQTTYLAQQYCHEAIREISKLRPSPERDALIQLSEIVLTRDK | |
Gene Information
Entrez Gene ID
Gene Name
prenyl (decaprenyl) diphosphate synthase, subunit 1
Gene Symbol
Species
Homo sapiens
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0005759 | TAS:Reactome | C | mitochondrial matrix |
| GO:0046872 | IEA:UniProtKB-KW | F | metal ion binding |
| GO:0046982 | IDA:HGNC | F | protein heterodimerization activity |
| GO:0000010 | IEA:Ensembl | F | trans-hexaprenyltranstransferase activity |
| GO:0050347 | IEA:Ensembl | F | trans-octaprenyltranstransferase activity |
| GO:0008299 | IDA:HGNC | P | isoprenoid biosynthetic process |
| GO:0051290 | IEA:Ensembl | P | protein heterotetramerization |
| GO:0044281 | TAS:Reactome | P | small molecule metabolic process |
| GO:0006744 | IDA:HGNC | P | ubiquinone biosynthetic process |
KEGG Pathway Links
| KEGG Pathway ID | Description |
|---|---|
| hsa00900 | Terpenoid backbone biosynthesis |
| ko00900 | Terpenoid backbone biosynthesis |
BIOCYC Pathway Links
| BIOCYC Pathway ID | Description |
|---|---|
| PWY-5806 | all-trans-decaprenyl diphosphate biosynthesis |
REACTOME Pathway Links
| REACTOME Pathway ID | Description |
|---|---|
| REACT_160111 | Ubiquinol biosynthesis |
Domain Information
UniProt Annotations
Entry Information
Gene Name
prenyl (decaprenyl) diphosphate synthase, subunit 1
Protein Entry
DPS1_HUMAN
UniProt ID
Species
Human
Comments
| Comment Type | Description |
|---|---|
| Alternative Products | Event=Alternative splicing; Named isoforms=3; Name=1; IsoId=Q5T2R2-1; Sequence=Displayed; Name=2; IsoId=Q5T2R2-2; Sequence=VSP_017101; Note=No experimental confirmation available.; Name=3; IsoId=Q5T2R2-3; Sequence=VSP_017100; |
| Catalytic Activity | (2E,6E)-farnesyl diphosphate + 7 isopentenyl diphosphate = 7 diphosphate + all-trans-decaprenyl diphosphate. |
| Cofactor | Name=Mg(2+); Xref=ChEBI |
| Disease | Coenzyme Q10 deficiency, primary, 2 (COQ10D2) [MIM |
| Function | Supplies decaprenyl diphosphate, the precursor for the side chain of the isoprenoid quinones ubiquinone-10. |
| Pathway | Cofactor biosynthesis; ubiquinone biosynthesis. |
| Similarity | Belongs to the FPP/GGPP synthase family. |
| Subcellular Location | Mitochondrion . |
| Subunit | Heterotetramer of 2 DPS1/TPRT and 2 DLP1 subunits. |
Identical and Related Proteins
Unique RefSeq proteins for LMP005824 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 50659086 | RefSeq | NP_055132 | 415 | decaprenyl-diphosphate synthase subunit 1 |
Identical Sequences to LMP005824 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:50659086 | DBBJ | BAE48216.1 | 415 | subunit 1 of decaprenyl diphosphate synthase [Homo sapiens] |
| GI:50659086 | GenBank | EAW86084.1 | 415 | prenyl (decaprenyl) diphosphate synthase, subunit 1, isoform CRA_a [Homo sapiens] |
| GI:50659086 | GenBank | AHD79539.1 | 415 | Sequence 29183 from patent US 8586006 |
| GI:50659086 | SwissProt | Q5T2R2.1 | 415 | RecName: Full=Decaprenyl-diphosphate synthase subunit 1; AltName: Full=All-trans-decaprenyl-diphosphate synthase subunit 1; AltName: Full=Decaprenyl pyrophosphate synthase subunit 1; AltName: Full=Trans-prenyltransferase 1; Short=TPT 1 [Homo sapiens] |
Related Sequences to LMP005824 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:50659086 | DBBJ | BAD97134.1 | 415 | trans-prenyltransferase variant, partial [Homo sapiens] |
| GI:50659086 | GenBank | AAH49211.1 | 414 | PDSS1 protein, partial [Homo sapiens] |
| GI:50659086 | GenBank | ACM82577.1 | 432 | Sequence 8075 from patent US 6812339 |
| GI:50659086 | GenBank | JAA00776.1 | 415 | prenyl (decaprenyl) diphosphate synthase, subunit 1 [Pan troglodytes] |
| GI:50659086 | GenBank | JAA27363.1 | 415 | prenyl (decaprenyl) diphosphate synthase, subunit 1 [Pan troglodytes] |
| GI:50659086 | RefSeq | XP_009456379.1 | 415 | PREDICTED: decaprenyl-diphosphate synthase subunit 1 [Pan troglodytes] |