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Information on EC 2.5.1.91 - all-trans-decaprenyl-diphosphate synthase and Organism(s) Homo sapiens and UniProt Accession Q86YH6

for references in articles please use BRENDA:EC2.5.1.91
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IUBMB Comments
This enzyme catalyses the condensation reactions resulting in the formation of all-trans-decaprenyl diphosphate, the isoprenoid side chain of ubiquinone-10 and menaquinone-10. The enzyme adds seven isopentenyl diphosphate molecules sequentially to farnesyl diphosphate with trans stereochemistry.
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This record set is specific for:
Homo sapiens
UNIPROT: Q86YH6
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Word Map
The taxonomic range for the selected organisms is: Homo sapiens
The expected taxonomic range for this enzyme is: Bacteria, Eukaryota
Synonyms
rsdds, decaprenyl diphosphate synthase gene, d-less polyprenyl diphosphate synthase, decaprenyl-pp synthase, ddsa protein, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
decaprenyl diphosphate synthase
-
PATHWAY SOURCE
PATHWAYS
-
-, -
SYSTEMATIC NAME
IUBMB Comments
(2E,6E)-farnesyl-diphosphate:isopentenyl-diphosphate farnesyltranstransferase (adding 7 isopentenyl units)
This enzyme catalyses the condensation reactions resulting in the formation of all-trans-decaprenyl diphosphate, the isoprenoid side chain of ubiquinone-10 and menaquinone-10. The enzyme adds seven isopentenyl diphosphate molecules sequentially to farnesyl diphosphate with trans stereochemistry.
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
subunit DLP1
UniProt
Manually annotated by BRENDA team
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
pathogenic mutations in PDSS2 (PDSS2 encodes the second subunit of decaprenyl diphosphate synthase) causing primary CoQ10 deficiency in an infant with fatal Leigh syndrome and nephrotic syndrome
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
DLP1_HUMAN
399
0
44129
Swiss-Prot
Mitochondrion (Reliability: 2)
SUBUNIT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
tetramer
2 * Dps1 + 2 * Dlp1, gel filtration and calculated
heterotetramer
heterotetramer of 2 DPS1 and 2 DLP1 subunits, both components are involved in determining the length of the ubiquinone side chain
tetramer
2 * Dps1 + 2 * Dlp1, gel filtration and calculated
PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
expression in Escherichia coli
expression in Escherichia coli
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Saiki, R.; Nagata, A.; Kainou, T.; Matsuda, H.; Kawamukai, M.
Characterization of solanesyl and decaprenyl diphosphate synthases in mice and humans
FEBS J.
272
5606-5622
2005
Homo sapiens (Q5T2R2), Homo sapiens (Q5T2R2 and Q86YH6), Homo sapiens (Q86YH6)
Manually annotated by BRENDA team
Lopez, L.C.; Schuelke, M.; Quinzii, C.M.; Kanki, T.; Rodenburg, R.J.; Naini, A.; Dimauro, S.; Hirano, M.
Leigh syndrome with nephropathy and CoQ10 deficiency due to decaprenyl diphosphate synthase subunit 2 (PDSS2) mutations
Am. J. Hum. Genet.
79
1125-1129
2006
Homo sapiens (Q5T2R2 and Q86YH6)
Manually annotated by BRENDA team