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Product Name COQ6 Chinese Name 辅酶生物合成单加氧酶COQ6抗体 Alias CGI-10; Coenzyme Q6 homolog (yeast); Coenzyme Q6 homolog, monooxygenase (S. cerevisiae); Coenzyme Q6 homolog, monooxygenase (yeast); coq6; COQ6_HUMAN; Ubiquinone biosynthesis monooxygenase COQ6. Research Area Cell biology Neurobiology Apoptosis Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, Dog, Pig, Horse, ) Applications
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 51kDa Cellular localization cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from mouse COQ6 : 351-450/468 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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]
Subcellular Location:
Golgi apparatus. Cell projection. Note=Localizes to podocyte cell processes.
Tissue Specificity:
Widely epressed.
DISEASE:
Coenzyme Q10 deficiency, primary, 6 (COQ10D6) [MIM:614650]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry.
Similarity:
Belongs to the ubiH/COQ6 family.
SWISS:
Q8R1S0
Gene ID:
217707
Database links:Entrez Gene: 51004 Human
SwissProt: Q9Y2Z9 Human
Unigene: 131555 Human
Product Picture Paraformaldehyde-fixed, paraffin embedded (human kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Incubation with (COQ6) Polyclonal Antibody, Unconjugated (SL25208R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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