CL640; Para-Hydroxybenzoate-Polyprenyltransferase,Mitochondrial; 4-hydroxybenzoate polyprenyltransferase, mitochondrial

For Research Use Only. Not for use in diagnostic procedures.

This assay has high sensitivity and excellent specificity for detection of Rat COQ2. No significant cross-reactivity or interference between Rat COQ2 and analogues was observed.

Store at 2-8 degree C.

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Small volumes of COQ2 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS285516 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the 4-hydroxybenzoate polyprenyltransferase (COQ2) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing COQ2. The ELISA analytical biochemical technique of the MBS285516 kit is based on COQ2 antibody-COQ2 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect COQ2 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, COQ2. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Principle of the Assay: This assay employs a two-site sandwich ELISA to quantitate COQ2 in samples. An antibody specific for COQ2 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyCOQ2 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for COQ2 is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of COQ2 bound in the initial step. The color development is stopped and the intensity of the color is measured.

40,406 Da

coenzyme Q2, polyprenyltransferase

4-hydroxybenzoate polyprenyltransferase, mitochondrial

4-hydroxybenzoate polyprenyltransferase, mitochondrial

4-HB polyprenyltransferase; PHB:PPT; PHB:polyprenyltransferase??[Similar Products]

COQ2: Catalyzes the prenylation of para-hydroxybenzoate (PHB) with an all-trans polyprenyl group. Mediates the second step in the final reaction sequence of coenzyme Q (CoQ) biosynthesis, which is the condensation of the polyisoprenoid side chain with PHB. Defects in COQ2 are the cause of coenzyme Q10 deficiency, primary, type 1 (COQ10D1). 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 UbiA prenyltransferase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Cofactor and Vitamin Metabolism - ubiquinone and other terpenoid-quinone biosynthesis; EC 2.5.1.39; Membrane protein, integral; Membrane protein, multi-pass; Mitochondrial; Transferase

Chromosomal Location of Human Ortholog: 14p22

Cellular Component: integral to mitochondrial inner membrane; mitochondrion

Molecular Function: 4-hydroxybenzoate decaprenyltransferase activity; 4-hydroxybenzoate nonaprenyltransferase activity

Biological Process: glycerol metabolic process; isoprenoid biosynthetic process; ubiquinone biosynthetic process

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