Decaprenyl-diphosphate synthase subunit 1 is an enzyme that in humans is encoded by the PDSS1 gene .[ 5] [ 6]
PDSS1 Identifiers Aliases PDSS1 External IDs OMIM : 607429 ; MGI : 1889278 ; HomoloGene : 5353 ; GeneCards : PDSS1 ; OMA :PDSS1 - orthologs Wikidata
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]
^ a b c GRCh38: Ensembl release 89: ENSG00000148459 – Ensembl , May 2017^ a b c GRCm38: Ensembl release 89: ENSMUSG00000026784 – Ensembl , May 2017^ "Human PubMed Reference:" . National Center for Biotechnology Information, U.S. National Library of Medicine .^ "Mouse PubMed Reference:" . National Center for Biotechnology Information, U.S. National Library of Medicine .^ 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 . ^ a b "Entrez Gene: PDSS1 prenyl (decaprenyl) diphosphate synthase, subunit 1" .
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