PDSS2

Decaprenyl-diphosphate synthase subunit 2 (PDSS2) is a protein that in humans is encoded by the PDSS2 gene.^[5]

PDSS2
Identifiers
Aliases	PDSS2, C6orf210, COQ10D3, DLP1, bA59I9.3, hDLP1, prenyl (decaprenyl) diphosphate synthase, subunit 2, decaprenyl diphosphate synthase subunit 2, COQ1B
External IDs	OMIM: 610564; MGI: 1918615; HomoloGene: 56885; GeneCards: PDSS2; OMA:PDSS2 - orthologs
Gene location (Human) Chr. Chromosome 6 (human)[1] Band 6q21 Start 107,152,562 bp[1] End 107,459,564 bp[1]
Gene location (Mouse) Chr. Chromosome 10 (mouse)[2] Band 10 B2|10 22.91 cM Start 43,097,482 bp[2] End 43,340,878 bp[2]
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in buccal mucosa cell kidney tubule secondary oocyte tibia glomerulus metanephric glomerulus gonad visceral pleura parietal pleura endothelial cell Top expressed in zygote secondary oocyte tibialis anterior muscle digastric muscle vastus lateralis muscle extraocular muscle gastrocnemius muscle myocardium of ventricle sternocleidomastoid muscle temporal muscle More reference expression data BioGPS n/a
Gene ontology Molecular function transferase activity trans-hexaprenyltranstransferase activity protein heterodimerization activity Cellular component transferase complex mitochondrial matrix mitochondrion cytosol Biological process ubiquinone biosynthetic process protein heterotetramerization regulation of body fluid levels isoprenoid biosynthetic process Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 57107 71365 Ensembl ENSG00000164494 ENSMUSG00000038240 UniProt Q86YH6 Q33DR3 RefSeq (mRNA) NM_020381 NM_001168289 NM_027772 RefSeq (protein) NP_065114 NP_001161761 NP_082048 Location (UCSC) Chr 6: 107.15 – 107.46 Mb Chr 10: 43.1 – 43.34 Mb PubMed search [3] [4]
Wikidata
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Function

The protein encoded by this gene is an enzyme that synthesizes 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.^[5]

Clinical significance

It may be associated with Coenzyme Q10 deficiency.^[6]

See also

• PDSS1