COQ6 cdna clone
COQ6 cDNA Clone
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: Oxidoreductase; EC 1.14.13.-; Cofactor and Vitamin Metabolism - ubiquinone and other terpenoid-quinone biosynthesis; Mitochondrial
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 #AAA1278872) is a cDNA Clone and is intended for research purposes only. The product is available for immediate purchase. The amino acid sequence is listed below: atggcggccc ggcttgtcag ccgatgcggg gctgtgcgtg cagctcccca cagcggcccg ctggtgtcct ggcgcaggtg gtccggcgcc tcaacagaca ccgtgtatga cgtggtggtg tcgggtggag gcctggtggg cgctgccatg gcctgtgcct tgggatatga tattcacttt catgacaaga aaatcctgtt gctcgaagca ggtccaaaga aagtactgga gaaattgtca gaaacttaca gcaacagggt cagctccatt tcccctggct ctgcaacgct tctcagtagt tttggtgcct gggaccatat ctgcaacatg agatacagag cctttcggcg aatgcaggtg tgggacgcct gctcagaggc cctgataatg tttgataagg ataatttaga tgacatgggc tatatcgtgg agaatgatgt catcatgcat gctctcacta agcagttgga ggctgtgtct gaccgagtga cggttctcta caggagcaaa gccattcgct atacctggcc ttgtccattt cctatggccg actccagccc ttgggttcat attaccctag gtgatggcag caccttccag accaaattgt tgataggtgc agatggtcac aactccggag tacggcaggc tgttggaatc cagaatgtga gctggaacta tgaccagtct gctgttgtgg ctactctgca tttatcagag gccacagaaa acaacgtagc ctggcagaga tttcttccct ctgggcctat tgctctgctc ccgctctcag acaccttgag ttccttggtt tggtccacgt cccatgaaca tgcagcagag ctagttagca tggatgagaa aaaatttgtg gatgccgtta actctgcctt ttggagtgat gctgaccaca cggacttcat cgacacagct ggtgccatgc tgcagtatgc tgtcagcctt ctgaagccca ctaaggtctc ggctcgccag ctgcccccaa gcgtagccag ggtggatgcc aaaagccgag ttctgtttcc tcttgggttg ggacatgctg ctgagtacgt caggcctcgg gtggcgctca ttggggatgc agcccacaga gtccatccgc ttgcaggaca gggtgtcaac atgggctttg gggatatctc cagcttggcc catcacctca gtacggcagc cttcaatggg aaggacttag gttccgtgag ccacctcaca ggttatgaaa cagaaagaca gcgtcacaac actgctcttc tggctgctac agacttacta aaaaggctct attctaccag tgcctccccg cttgtgttgc tcaggacgtg gggcttgcag gccacaaatg cagtgtctcc actcaaagaa cagattatgg cctttgcaag caaatga. It is sometimes possible for the material contained within the vial of "COQ6, cDNA Clone" 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
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