Rabbit Anti-COQ6/FITC Conjugated antibody

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CGI-10; Coenzyme Q6 homolog (yeast); Coenzyme Q6 homolog, monooxygenase (S. cerevisiae); Coenzyme Q6 homolog, monooxygenase (yeast); coq6; COQ6_HUMAN; Ubiquinone biosynthesis monooxygenase COQ6.

Cat:

SL13987R-FITC

Species Reactivity：

Human,(predicted: Mouse,Rat,Rabbit,)

Immunogen：

KLH conjugated synthetic peptide derived from human COQ6

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

51kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
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.

Database links:

Entrez Gene: 51004 Human

SwissProt: Q9Y2Z9 Human

Unigene: 131555 Human

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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