Rabbit anti-Human COQ6 Polyclonal Antibody | anti-COQ6 antibody
COQ6 Antibody, FITC conjugated
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: Cofactor and Vitamin Metabolism - ubiquinone and other terpenoid-quinone biosynthesis; EC 1.14.13.-; Mitochondrial; Oxidoreductase
Chromosomal Location of Human Ortholog: 14q24.3
Cellular Component: mitochondrion
Molecular Function: monooxygenase activity
Biological Process: ubiquinone biosynthetic process
Disease: Coenzyme Q10 Deficiency, Primary, 6
Research Articles on COQ6
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Product Notes
The COQ6 coq6 (Catalog #AAA7051666) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The COQ6 Antibody, FITC conjugated reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's COQ6 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA). Researchers should empirically determine the suitability of the COQ6 coq6 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "COQ6, Polyclonal Antibody" 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
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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