Human coenzyme Q6 homolog, monooxygenase (S. cerevisiae) ELISA Kit | COQ6 elisa kit
Human Ubiquinone biosynthesis monooxygenase COQ6, COQ6 ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene belongs to the ubiH/COQ6 family. It is an evolutionarily conserved monooxygenase required for the biosynthesis of coenzyme Q10 (or ubiquinone), which is an essential component of the mitochondrial electron transport chain, and one of the most potent lipophilic antioxidants implicated in the protection of cell damage by reactive oxygen species. Knockdown of this gene in mouse and zebrafish results in decreased growth due to increased apoptosis. Mutations in this gene are associated with autosomal recessive coenzyme Q10 deficiency-6 (COQ10D6), which manifests as nephrotic syndrome with sensorineural deafness. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Jun 2012]
Uniprot Description
COQ6: Defects in COQ6 are the cause of coenzyme Q10 deficiency, primary, type 6 (COQ10D6). An autosomal recessive disorder characterized by onset in infancy of severe progressive nephrotic syndrome resulting in end-stage renal failure and sensorineural deafness. Renal biopsy usually shows focal segmental glomerulosclerosis. Belongs to the UbiH/COQ6 family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 1.14.13.-; Cofactor and Vitamin Metabolism - ubiquinone and other terpenoid-quinone biosynthesis; Mitochondrial; Oxidoreductase
Chromosomal Location of Human Ortholog: 14q24.3
Cellular Component: Golgi apparatus; cell projection; mitochondrion
Molecular Function: oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NADH or NADPH as one donor, and incorporation of one atom of oxygen; FAD binding; monooxygenase activity
Biological Process: ubiquinone biosynthetic process
Disease: Coenzyme Q10 Deficiency, Primary, 6
Research Articles on COQ6
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Product Notes
The Human COQ6 coq6 (Catalog #AAA9342147) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9342147 ELISA Kit recognizes Human COQ6. It is sometimes possible for the material contained within the vial of "coenzyme Q6 homolog, monooxygenase (S. cerevisiae), 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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