COQ4 Antibody, FITC conjugated

Datasheet

Code	CSB-PA896885LC01HU
Size	US$166
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Product Details
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Target Background

Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) COQ4 Polyclonal antibody

Uniprot No.

Q9Y3A0

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

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.

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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

The COQ4 mutation was CRISPR/Cas9 edited resulting in isogenic, diploid and off-target free COQ4-corrected iPSCs. PMID: 28465093
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
five recessive missense mutations in COQ4 segregating with lethal neonatal mitochondrial encephalomyopathy in four families of Ashkenzi Jews PMID: 26185144
COQ4 mutations cause a broad spectrum of mitochondrial disorders associated with CoQ10 deficiency. PMID: 25658047
Haploinsufficiency in COQ4 resulted in reduced COQ4 expression, CoQ10 content and biosynthetic rate, and activities of respiratory chain complex II+III. PMID: 22368301
Human ortholog of Saccharomyces cerevisiae COQ4 was cloned and characterized. PMID: 18474229
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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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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