Human prenyl (decaprenyl) diphosphate synthase, subunit 2 ELISA Kit | PDSS2 elisa kit
Human Decaprenyl-diphosphate synthase subunit 2, PDSS2 ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
PDSS2: Supplies decaprenyl diphosphate, the precursor for the side chain of the isoprenoid quinones ubiquinone-10. Defects in PDSS2 are the cause of coenzyme Q10 deficiency, primary, type 3 (COQ10D3). A fatal encephalomyopathic form of coenzyme Q10 deficiency with nephrotic syndrome. Coenzyme Q10 deficiency is an autosomal recessive disorder with variable manifestations consistent with 5 major phenotypes. The phenotypes include an encephalomyopathic form with seizures and ataxia; a multisystem infantile form with encephalopathy, cardiomyopathy and renal failure; a predominantly cerebellar form with ataxia and cerebellar atrophy; Leigh syndrome with growth retardation; and an isolated myopathic form. Belongs to the FPP/GGPP synthase family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 2.5.1.91; Secondary Metabolites Metabolism - terpenoid backbone biosynthesis; Transferase
Chromosomal Location of Human Ortholog: 6q21
Cellular Component: mitochondrial matrix; cytoplasm
Molecular Function: protein heterodimerization activity; trans-hexaprenyltranstransferase activity; trans-octaprenyltranstransferase activity
Biological Process: ubiquinone biosynthetic process; regulation of body fluid levels; isoprenoid biosynthetic process; protein heterotetramerization
Disease: Coenzyme Q10 Deficiency, Primary, 3
Research Articles on PDSS2
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Product Notes
The Human PDSS2 pdss2 (Catalog #AAA9328978) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9328978 ELISA Kit recognizes Human PDSS2. It is sometimes possible for the material contained within the vial of "prenyl (decaprenyl) diphosphate synthase, subunit 2, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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