Rabbit anti-Human PDSS2 Polyclonal Antibody

Uniprot Id

Q86YH6

Target Species

Human

Target Name

PDSS2

Target Full Name

All trans-polyprenyl-diphosphate synthase PDSS2

Target Function

Heterotetrameric enzyme that catalyzes the condensation of farnesyl diphosphate (FPP), which acts as a primer, and isopentenyl diphosphate (IPP) to produce prenyl diphosphates of varying chain lengths and participates in the determination of the side chain of ubiquinone. Supplies nona and decaprenyl diphosphate, the precursors for the side chain of the isoprenoid quinones ubiquinone-9 (Q9) and ubiquinone-10 (Q10) respectively. The enzyme adds isopentenyl diphosphate molecules sequentially to farnesyl diphosphate with trans stereochemistry. May play a role during cerebellar development. May regulate mitochondrial respiratory chain function.

Target Involvement

Coenzyme Q10 deficiency, primary, 3 (COQ10D3)

Target Subcellular Location

Mitochondrion.

Target Protein Families

FPP/GGPP synthase family

Target Synonyms

All-trans-decaprenyl-diphosphate synthase subunit 2; bA59I9.3; C6orf210; Candidate tumor suppressor protein; chromosome 6 open reading frame 210; Decaprenyl pyrophosphate synthase subunit 2; decaprenyl pyrophosphate synthetase subunit 2 ; Decaprenyl-diphosphate synthase subunit 2; DLP1; DLP1_HUMAN; hDLP1; Pdss2; prenyl (decaprenyl) diphosphate synthase; subunit 2; subunit 2 of decaprenyl diphosphate synthase

Target Background

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.