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

Store all reagents at 2-8 degree C.

We suggest pre-experimenting with neat (undiluted) samples, 1:2 or 1:4 dilutions. Please avoid diluting your samples more than 1:10 as it would exceed the dilution limit set for this kit. If the expected concentration of the target is beyond the detection range of the kit, please contact our technical support team

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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.

MBS7206729 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the 4-hydroxybenzoate polyprenyltransferase, mitochondrial (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 MBS7206729 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(%).

35,468 Da

coenzyme Q2 4-hydroxybenzoate polyprenyltransferase

4-hydroxybenzoate polyprenyltransferase, mitochondrial; PHB:polyprenyltransferase; coenzyme Q2 homolog, prenyltransferase; para-hydroxybenzoate-polyprenyltransferase, mitochondrial

4-hydroxybenzoate polyprenyltransferase, mitochondrial

CL640; hCOQ2; PHB:polyprenyltransferase??[Similar Products]

COQ2_HUMAN

This gene encodes an enzyme that functions in the final steps in the biosynthesis of CoQ (ubiquinone), a redox carrier in the mitochondrial respiratory chain and a lipid-soluble antioxidant. This enzyme, which is part of the coenzyme Q10 pathway, catalyzes the prenylation of parahydroxybenzoate with an all-trans polyprenyl group. Mutations in this gene cause coenzyme Q10 deficiency, a mitochondrial encephalomyopathy, and also COQ2 nephropathy, an inherited form of mitochondriopathy with primary renal involvement. [provided by RefSeq, Oct 2009]

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

Chromosomal Location of Human Ortholog: 4q21.23

Cellular Component: mitochondrial inner membrane; integral to membrane

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

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

Disease: Multiple System Atrophy 1, Susceptibility To; Coenzyme Q10 Deficiency, Primary, 1

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