COQ6 Antibody

Datasheet

Code	CSB-PA005805GA01HU
Size	$600
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Product Details
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Target Background

Product Details

Uniprot No.

Q9Y2Z9

Target Names

COQ6

Alternative Names

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

Raised in

Rabbit

Species Reactivity

Human,Mouse,Rat

Immunogen

Human COQ6

Immunogen Species

Homo sapiens (Human)

Isotype

IgG

Purification Method

Antigen Affinity Purified

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.

Tested Applications

ELISA,WB

Protocols

ELISA Protocol
Western Blotting(WB) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Customer Reviews and Q&A

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Target Background

Function

FAD-dependent monooxygenase required for the C5-ring hydroxylation during ubiquinone biosynthesis. Catalyzes the hydroxylation of 3-hexaprenyl-4-hydroxybenzoic acid (HHB) to 3-hexaprenyl-4,5-dihydroxybenzoic acid (DHHB). The electrons required for the hydroxylation reaction may be funneled indirectly from NADPH via a ferredoxin/ferredoxin reductase system to COQ6. Is able to perform the deamination reaction at C4 of 3-hexaprenyl-4-amino-5-hydroxybenzoic acid (HHAB) to produce DHHB when expressed in yeast cells lacking COQ9, even if utilization of para-aminobenzoic acid (pABA) involving C4-deamination seems not to occur in bacteria, plants and mammals, where only C5 hydroxylation of HHB has been shown.

Gene References into Functions

Primary CoQ10 deficiency due to COQ6 mutations should be considered in children presenting with both Steroid-Resistant Focal Segmental Glomerulosclerosis and sensorineural hearing loss. An early diagnosis of COQ6 mutations is essential because the condition is treatable when CoQ10 supplementation is started at the early stage. PMID: 28117207
Data indicate a heterozygous loss-of-function coenzyme Q10 (CoQ10) biosynthesis monooxygenase 6 gene (COQ6)missense mutation in familial schwannomatosis. PMID: 24763291
COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness PMID: 21540551

Involvement in disease

Coenzyme Q10 deficiency, primary, 6 (COQ10D6)

Subcellular Location

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side. Golgi apparatus. Cell projection.

Protein Families

UbiH/COQ6 family

Tissue Specificity

Widely expressed.

Database Links

HGNC: 20233

OMIM: 614647

KEGG: hsa:51004

STRING: 9606.ENSP00000333946

UniGene: Hs.131555

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Antibody FAQs What Is an Antibody? How to Choose an Antibody for Scientific Research? The difference between primary antibody and secondary antibod The Meaning of Irregular Antibody Screening Tag Antibodies, Your Good Partner in Protein Research How to Choose the Loading Control Antibodies?

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301-363-4651 (Available 9 a.m. to 5 p.m. CST from Monday to Friday)

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Address

7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA

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