PDSS1

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. Defects in this gene are a cause of coenzyme Q10 deficiency. [provided by RefSeq, Jul 2008]

Description

decaprenyl diphosphate synthase subunit 1

Protein family

Disease related genes, Enzymes, Mapped to neXtProt, Mapped to UniProt SWISS-PROT, Potential drug targets, Predicted intracellular proteins, Protein evidence (Peptideatlas)

Chromosome

10:p12.1 (26697701 - 26746798)

Ensembl ID

ENSG00000148459  (ver. 99.38)

Orthologs

Mouse Pdss1: ENSMUSG00000026784

UniProt

Q5T2R2

neXtProt

NX_Q5T2R2

Human splice variants
Mouse splice variants