Aliases for PDSS2 Gene
External Ids for PDSS2 Gene
Previous HGNC Symbols for PDSS2 Gene
Previous GeneCards Identifiers for PDSS2 Gene
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. Defects in this gene are a cause of coenzyme Q10 deficiency.[provided by RefSeq, Oct 2009]
GeneCards Summary for PDSS2 Gene
PDSS2 (Decaprenyl Diphosphate Synthase Subunit 2) is a Protein Coding gene. Diseases associated with PDSS2 include Coenzyme Q10 Deficiency, Primary, 3 and Leigh Syndrome With Nephrotic Syndrome. Among its related pathways are Metabolism and Terpenoid backbone biosynthesis. Gene Ontology (GO) annotations related to this gene include protein heterodimerization activity and trans-octaprenyltranstransferase activity. An important paralog of this gene is PDSS1.
UniProtKB/Swiss-Prot Summary for PDSS2 Gene
Supplies decaprenyl diphosphate, the precursor for the side chain of the isoprenoid quinones ubiquinone-10.