PDSS2

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

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.

Clinical significance

It may be associated with Coenzyme Q10 deficiency.

See also

  • PDSS1
  • References

    Further reading

    This article incorporates text from the United States National Library of Medicine, which is in the public domain.

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