PDSS1

Protein-coding gene in the species Homo sapiens
PDSS1
Identifiers
AliasesPDSS1, COQ1, COQ10D2, DPS, SPS, TPRT, TPT, TPT 1, hDPS1, prenyl (decaprenyl) diphosphate synthase, subunit 1, decaprenyl diphosphate synthase subunit 1, COQ1A
External IDsOMIM: 607429; MGI: 1889278; HomoloGene: 5353; GeneCards: PDSS1; OMA:PDSS1 - orthologs
Gene location (Human)
Chromosome 10 (human)
Chr.Chromosome 10 (human)[1]
Chromosome 10 (human)
Genomic location for PDSS1
Genomic location for PDSS1
Band10p12.1Start26,697,701 bp[1]
End26,746,798 bp[1]
Gene location (Mouse)
Chromosome 2 (mouse)
Chr.Chromosome 2 (mouse)[2]
Chromosome 2 (mouse)
Genomic location for PDSS1
Genomic location for PDSS1
Band2|2 A3Start22,785,534 bp[2]
End22,830,278 bp[2]
RNA expression pattern
Bgee
HumanMouse (ortholog)
Top expressed in
  • rectum

  • jejunal mucosa

  • duodenum

  • monocyte

  • cerebellar hemisphere

  • ganglionic eminence

  • gastrocnemius muscle

  • Achilles tendon

  • upper lobe of left lung

  • right lobe of liver
Top expressed in
  • Paneth cell

  • proximal tubule

  • otic placode

  • saccule

  • soleus muscle

  • duodenum

  • jejunum

  • yolk sac

  • myocardium of ventricle

  • atrioventricular valve
More reference expression data
BioGPS
More reference expression data
Gene ontology
Molecular function
  • metal ion binding
  • protein heterodimerization activity
  • trans-hexaprenyltranstransferase activity
  • transferase activity
Cellular component
  • mitochondrial matrix
  • mitochondrion
  • transferase complex
Biological process
  • isoprenoid biosynthetic process
  • protein heterotetramerization
  • ubiquinone biosynthetic process
Sources:Amigo / QuickGO
Orthologs
SpeciesHumanMouse
Entrez

23590

56075

Ensembl

ENSG00000148459

ENSMUSG00000026784

UniProt

Q5T2R2

Q33DR2

RefSeq (mRNA)

NM_014317
NM_001321978
NM_001321979

NM_019501
NM_001355467

RefSeq (protein)

NP_001308907
NP_001308908
NP_055132

NP_062374
NP_001342396

Location (UCSC)Chr 10: 26.7 – 26.75 MbChr 2: 22.79 – 22.83 Mb
PubMed search[3][4]
Wikidata
View/Edit HumanView/Edit Mouse

Decaprenyl-diphosphate synthase subunit 1 is an enzyme that in humans is encoded by the PDSS1 gene.[5][6]

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.[6]

References

  1. ^ a b c GRCh38: Ensembl release 89: ENSG00000148459 – Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000026784 – Ensembl, May 2017
  3. ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. ^ Rotig A, Appelkvist EL, Geromel V, Chretien D, Kadhom N, Edery P, Lebideau M, Dallner G, Munnich A, Ernster L, Rustin P (Sep 2000). "Quinone-responsive multiple respiratory-chain dysfunction due to widespread coenzyme Q10 deficiency". Lancet. 356 (9227): 391–395. doi:10.1016/S0140-6736(00)02531-9. PMID 10972372. S2CID 27320145.
  6. ^ a b "Entrez Gene: PDSS1 prenyl (decaprenyl) diphosphate synthase, subunit 1".

Further reading

  • Appelkvist EL, Aberg F, Guan Z, et al. (1995). "Regulation of coenzyme Q biosynthesis". Mol. Aspects Med. 15 Suppl: s37–46. doi:10.1016/0098-2997(94)90011-6. PMID 7752843.
  • Runquist M, Ericsson J, Thelin A, et al. (1994). "Isoprenoid biosynthesis in rat liver mitochondria. Studies on farnesyl pyrophosphate synthase and trans-prenyltransferase". J. Biol. Chem. 269 (8): 5804–9. doi:10.1016/S0021-9258(17)37533-6. PMID 8119922.
  • Maruyama K, Sugano S (1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides". Gene. 138 (1–2): 171–174. doi:10.1016/0378-1119(94)90802-8. PMID 8125298.
  • Teclebrhan H, Olsson J, Swiezewska E, Dallner G (1993). "Biosynthesis of the side chain of ubiquinone:trans-prenyltransferase in rat liver microsomes". J. Biol. Chem. 268 (31): 23081–6. doi:10.1016/S0021-9258(19)49428-3. PMID 8226825.
  • Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, et al. (1997). "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library". Gene. 200 (1–2): 149–156. doi:10.1016/S0378-1119(97)00411-3. PMID 9373149.
  • Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–16903. Bibcode:2002PNAS...9916899M. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932.
  • Deloukas P, Earthrowl ME, Grafham DV, et al. (2004). "The DNA sequence and comparative analysis of human chromosome 10". Nature. 429 (6990): 375–381. Bibcode:2004Natur.429..375D. doi:10.1038/nature02462. PMID 15164054.
  • Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–2127. doi:10.1101/gr.2596504. PMC 528928. PMID 15489334.
  • Saiki R, Nagata A, Kainou T, et al. (2005). "Characterization of solanesyl and decaprenyl diphosphate synthases in mice and humans". FEBS J. 272 (21): 5606–5622. doi:10.1111/j.1742-4658.2005.04956.x. PMID 16262699. S2CID 13735213.
  • Mollet J, Giurgea I, Schlemmer D, et al. (2007). "Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders". J. Clin. Invest. 117 (3): 765–772. doi:10.1172/JCI29089. PMC 1804361. PMID 17332895.
  • v
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CycleAnaplerotic
to acetyl-CoA
to α-ketoglutaric acid
to succinyl-CoA
to oxaloacetic acid
Mitochondrial
electron transport chain/
oxidative phosphorylation
Primary
Other


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