• Gene id: 23590

Gene Summary

• Gene Symbol: PDSS1   Gene   UCSC   Ensembl
• Aliases: COQ1, COQ10D2, COQ1A, DPS, SPS, TPRT, TPT, TPT 1, hDPS1
• Gene name: decaprenyl diphosphate synthase subunit 1
• Alternate names: decaprenyl-diphosphate synthase subunit 1, all-trans-decaprenyl-diphosphate synthase subunit 1, coenzyme Q1 homolog, decaprenyl pyrophosphate synthase subunit 1, polyprenyl pyrophosphate synthetase, prenyl (decaprenyl) diphosphate synthase, subunit 1, subunit 1,
• Gene location: 10p12.1 (26697423: 26747245)     Exons: 14     NC_000010.11
• Gene summary(Entrez): 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 isopent
• OMIM: 601683

Protein Summary

• Protein general information: Q5T2R2  
  • Name: Decaprenyl diphosphate synthase subunit 1 (EC 2.5.1.91) (All trans decaprenyl diphosphate synthase subunit 1) (Decaprenyl pyrophosphate synthase subunit 1) (Trans prenyltransferase 1) (TPT 1)
  • Length: 415  
  • Mass: 46261
• Sequence:

  MASRWWRWRRGCSWKPAARSPGPGSPGRAGPLGPSAAAEVRAQVHRRKGLDLSQIPYINLVKHLTSACPNVCRIS
  RFHHTTPDSKTHSGEKYTDPFKLGWRDLKGLYEDIRKELLISTSELKEMSEYYFDGKGKAFRPIIVALMARACNI
  HHNNSRHVQASQRAIALIAEMIHTASLVHDDVIDDASSRRGKHTVNKIWGEKKAVLAGDLILSAASIALARIGNT
  TVISILTQVIEDLVRGEFLQLGSKENENERFAHYLEKTFKKTASLIANSCKAVSVLGCPDPVVHEIAYQYGKNVG
  IAFQLIDDVLDFTSCSDQMGKPTSADLKLGLATGPVLFACQQFPEMNAMIMRRFSLPGDVDRARQYVLQSDGVQQ
  TTYLAQQYCHEAIREISKLRPSPERDALIQLSEIVLTRDK

• Structural information:
  • Interpro: IPR008949  IPR000092  IPR033749  
  • Prosite: PS00723 PS00444
  • STRING: ENSP00000365388
• Other Databases: GeneCards:  PDSS1  Malacards:  PDSS1

Gene ontology

GO accession	Term name	Evidence code	Go category
GO:0008299	isoprenoid biosynthetic process	IDA	biological process
GO:0006744	ubiquinone biosynthetic process	IDA	biological process
GO:0000010	trans-hexaprenyltranstransferase activity	IDA	contributes to
GO:0005515	protein binding	IPI	molecular function
GO:0004659	prenyltransferase activity	IBA	molecular function
GO:0005739	mitochondrion	IBA	cellular component
GO:0006744	ubiquinone biosynthetic process	IBA	biological process
GO:0008299	isoprenoid biosynthetic process	IBA	biological process
GO:1990234	transferase complex	IBA	cellular component
GO:0008299	isoprenoid biosynthetic process	IEA	biological process
GO:0016740	transferase activity	IEA	molecular function
GO:0008299	isoprenoid biosynthetic process	IEA	biological process
GO:0046872	metal ion binding	IEA	molecular function
GO:0005739	mitochondrion	IEA	cellular component
GO:0006744	ubiquinone biosynthetic process	IEA	biological process
GO:0005759	mitochondrial matrix	TAS	cellular component
GO:0006744	ubiquinone biosynthetic process	TAS	biological process
GO:0050347	trans-octaprenyltranstransferase activity	IEA	molecular function
GO:0046982	protein heterodimerization activity	IEA	molecular function
GO:0006744	ubiquinone biosynthetic process	IEA	biological process
GO:0000010	trans-hexaprenyltranstransferase activity	IEA	molecular function
GO:1990234	transferase complex	IEA	cellular component
GO:0008299	isoprenoid biosynthetic process	IEA	biological process
GO:1990234	transferase complex	IDA	cellular component
GO:0005739	mitochondrion	IEA	cellular component
GO:0006744	ubiquinone biosynthetic process	IEA	biological process

KEGG pathways

Pathway id	Pathway name
hsa00900	Terpenoid backbone biosynthesis

Diseases

Associated diseases	References
Coenzyme Q10 deficiency	KEGG:H00999
Coenzyme Q10 deficiency	KEGG:H00999
Spermatogenic defects	MIK: 31037746

PubMed references

PMID	Condition	Mutation	Ethnicity	Population details	Infertility_type	Associated_genes
31037746	Spermatogenic defects			28 men with azoospermia	Male infertility	Microarray