COQ4 Antibody, FITC conjugated

說明書

中文名稱：

COQ4兔多克隆抗體, FITC偶聯(lián)
貨號：

CSB-PA896885LC01HU
規(guī)格：

￥880
其他：

產(chǎn)品詳情
產(chǎn)品評價
相關(guān)產(chǎn)品
靶點詳情

產(chǎn)品詳情

產(chǎn)品名稱：

Rabbit anti-Homo sapiens (Human) COQ4 Polyclonal antibody
Uniprot No.：

Q9Y3A0
基因名：

COQ4
別名：

CGI 92 antibody; Coenzyme Q biosynthesis protein 4 homolog antibody; coq4 antibody; COQ4_HUMAN antibody; mitochondrial antibody; Ubiquinone biosynthesis protein COQ4 homolog antibody
宿主：

Rabbit
反應(yīng)種屬：

Human
免疫原：

Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial protein (31-265AA)
免疫原種屬：

Homo sapiens (Human)
標(biāo)記方式：

FITC
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
產(chǎn)品提供形式：

Liquid
儲存條件：

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

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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評價

靶點詳情

功能：

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.
基因功能參考文獻(xiàn)：
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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相關(guān)疾?。?/div>
Coenzyme Q10 deficiency, primary, 7 (COQ10D7)
亞細(xì)胞定位：

[Isoform 1]: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
蛋白家族：

COQ4 family
組織特異性：

Expressed ubiquitously, but at high levels in liver, lung and pancreas.
數(shù)據(jù)庫鏈接：

HGNC: 19693

OMIM: 612898

KEGG: hsa:51117

STRING: 9606.ENSP00000300452

UniGene: Hs.98541

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