COQ6 Antibody

Code CSB-PA897098LA01HU
Size US$166
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  • Western Blot
    Positive WB detected in: Mouse heart tissue, Mouse liver tissue
    All lanes: COQ6 antibody at 3μg/ml
    Secondary
    Goat polyclonal to rabbit IgG at 1/50000 dilution
    predicted band size: 51, 49, 43 kDa
    observed band size: 51, 49 kDa

  • Immunohistochemistry of paraffin-embedded human testis tissue using CSB-PA897098LA01HU at dilution of 1:100

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) COQ6 Polyclonal antibody
Uniprot No.
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
Immunogen
Recombinant Human Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial protein (208-339AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The COQ6 Antibody (Product code: CSB-PA897098LA01HU) is Non-conjugated. For COQ6 Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA897098LB01HU COQ6 Antibody, HRP conjugated ELISA
FITC CSB-PA897098LC01HU COQ6 Antibody, FITC conjugated
Biotin CSB-PA897098LD01HU COQ6 Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Tested Applications
ELISA, WB, IHC
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:5000
IHC 1:20-1:200
Troubleshooting and 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.

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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
  1. 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
  2. Data indicate a heterozygous loss-of-function coenzyme Q10 (CoQ10) biosynthesis monooxygenase 6 gene (COQ6)missense mutation in familial schwannomatosis. PMID: 24763291
  3. 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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