Recombinant Full Length Human COQ6 Protein, GST-tagged

• Cat.No.: COQ6-1977HF
• Product Overview: Human COQ6 full-length ORF ( AAH14181, 1 a.a. - 468 a.a.) recombinant protein with GST-tag at N-terminal.

Specification

• 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]
• Source: In Vitro Cell Free System
• Species: Human
• Tag: GST
• Molecular Mass: 77.22 kDa
• Protein length: 468 amino acids
• AA Sequence: MAARLVSRCGAVRAAPHSGPLVSWRRWSGASTDTVYDVVVSGGGLVGAAMACALGYDIHFHDKKILLLEAGPKKVLEKLSETYSNRVSSISPGSATLLSSFGAWDHICNMRYRAFRRMQVWDACSEALIMFDKDNLDDMGYIVENDVIMHALTKQLEAVSDRVTVLYRSKAIRYTWPCPFPMADSSPWVHITLGDGSTFQTKLLIGADGHNSGVRQAVGIQNVSWNYDQSAVVATLHLSEATENNVAWQRFLPSGPIALLPLSDTLSSLVWSTSHEHAAELVSMDEEKFVDAVNSAFWSDADHTDFIDTAGAMLQYAVSLLKPTKVSARQLPPSVARVDAKSRVLFPLGLGHAAEYVRPRVALIGDAAHRVHPLAGQGVNMGFGDISSLAHHLSTAAFNGKDLGSVSHLTGYETERQRHNTALLAATDLLKRLYSTSASPLVLLRTWGLQATNAVSPLKEQIMAFASK
• Applications: Enzyme-linked Immunoabsorbent Assay
  Western Blot (Recombinant protein)
  Antibody Production
  Protein Array
• Notes: Best use within three months from the date of receipt of this protein.
• Storage: Store at -80 centigrade. Aliquot to avoid repeated freezing and thawing.
• Storage Buffer: 50 mM Tris-HCl, 10 mM reduced Glutathione, pH=8.0 in the elution buffer.

Gene Information

• Gene Name: COQ6 coenzyme Q6, monooxygenase [ Homo sapiens (human) ]
• Official Symbol: COQ6
• Synonyms: COQ6; coenzyme Q6, monooxygenase; Coenzyme Q6, Monooxygenase; Coenzyme Q10 Monooxygenase 6; Ubiquinone Biosynthesis Monooxygenase COQ6, Mitochondrial; Coenzyme Q6 Homolog, Monooxygenase (S. Cerevisiae); Coenzyme Q6 Homolog, Monooxygenase (Yeast); Coenzyme Q6 Homolog, Monooxygenase; Coenzyme Q6 Homolog (Yeast); Coenzyme Q6 Monooxygenase; EC 1.14.13.-; EC 1.14.13; COQ10D6; CGI-10; CGI10; ubiquinone biosynthesis monooxygenase COQ6, mitochondrial; coenzyme Q10 monooxygenase 6; coenzyme Q6 homolog, monooxygenase
• Gene ID: 51004
• mRNA Refseq: NM_182476
• Protein Refseq: NP_872282
• MIM: 614647
• UniProt ID: Q9Y2Z9