PDSS1

PDSS1
Identifiers
Aliases	PDSS1, COQ1, COQ10D2, DPS, SPS, TPRT, TPT, TPT 1, hDPS1, prenyl (decaprenyl) diphosphate synthase, subunit 1, decaprenyl diphosphate synthase subunit 1, COQ1A
External IDs	OMIM: 607429; MGI: 1889278; HomoloGene: 5353; GeneCards: PDSS1; OMA:PDSS1 - orthologs
Gene location (Human)
Chr.	Chromosome 10 (human)
End	26,746,798 bp
Gene location (Mouse)
Chr.	Chromosome 2 (mouse)
End	22,830,278 bp
RNA expression pattern
	Top expressed in
	mucosa of transverse colon; ; rectum; ; jejunal mucosa; ; duodenum; ; monocyte; ; testicle; ; cerebellar hemisphere; ; gonad; ; mucosa of ileum; ; right hemisphere of cerebellum;
	Top expressed in
	Paneth cell; ; proximal tubule; ; right kidney; ; otic placode; ; otic vesicle; ; saccule; ; soleus muscle; ; duodenum; ; jejunum; ; embryo;
	More reference expression data
	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
	23590
	56075
	ENSG00000148459
	ENSMUSG00000026784
	Q5T2R2
	Q33DR2
	NM_014317; NM_001321978; NM_001321979
	NM_019501; NM_001355467
	NP_001308907; NP_001308908; NP_055132
	NP_062374; NP_001342396
	Wikidata
View/Edit Human	View/Edit Mouse

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

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.