COQ4 Antibody, FITC conjugated

Code CSB-PA896885LC01HU
Size US$166
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) COQ4 Polyclonal antibody
Uniprot No.
Target Names
COQ4
Alternative Names
CGI 92 antibody; Coenzyme Q biosynthesis protein 4 homolog antibody; coq4 antibody; COQ4_HUMAN antibody; mitochondrial antibody; Ubiquinone biosynthesis protein COQ4 homolog antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial protein (31-265AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
FITC
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
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.
Usage
For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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Target Background

Function
Component of the coenzyme Q biosynthetic pathway. May play a role in organizing a multi-subunit COQ enzyme complex required for coenzyme Q biosynthesis. Required for steady-state levels of other COQ polypeptides.
Gene References into Functions
  1. The COQ4 mutation was CRISPR/Cas9 edited resulting in isogenic, diploid and off-target free COQ4-corrected iPSCs. PMID: 28465093
  2. Three genes in our epilepsy cohort (COQ4, DNM1, and PURA), accounting for 14% (3/21) of all novel genetic etiologies identified in patients with epilepsy, were subsequently confirmed in independent publications. PMID: 26795593
  3. five recessive missense mutations in COQ4 segregating with lethal neonatal mitochondrial encephalomyopathy in four families of Ashkenzi Jews PMID: 26185144
  4. COQ4 mutations cause a broad spectrum of mitochondrial disorders associated with CoQ10 deficiency. PMID: 25658047
  5. Haploinsufficiency in COQ4 resulted in reduced COQ4 expression, CoQ10 content and biosynthetic rate, and activities of respiratory chain complex II+III. PMID: 22368301
  6. Human ortholog of Saccharomyces cerevisiae COQ4 was cloned and characterized. PMID: 18474229
  7. homologous to Coq4p in S.cerevisiae PMID: 11469793

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Involvement in disease
Coenzyme Q10 deficiency, primary, 7 (COQ10D7)
Subcellular Location
[Isoform 1]: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
Protein Families
COQ4 family
Tissue Specificity
Expressed ubiquitously, but at high levels in liver, lung and pancreas.
Database Links

HGNC: 19693

OMIM: 612898

KEGG: hsa:51117

STRING: 9606.ENSP00000300452

UniGene: Hs.98541

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