COQ8A Antibody

說明書

中文名稱：

COQ8A兔多克隆抗體
貨號：

CSB-PA850918ESR1HU
規格：

￥440
圖片：
Immunohistochemistry of paraffin-embedded human colon tissue using CSB-PA850918ESR1HU at dilution of 1:100

Immunohistochemistry of paraffin-embedded human pancreatic tissue using CSB-PA850918ESR1HU at dilution of 1:100
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) COQ8A Polyclonal antibody
Uniprot No.：

Q8NI60
基因名：

COQ8A
別名：

aarF domain containing protein kinase 3 antibody; aarF domain-containing protein kinase 3 antibody; ADCK 3 antibody; Adck3 antibody; ADCK3_HUMAN antibody; ARCA2 antibody; CABC 1 antibody; CABC1 antibody; Chaperone ABC1 (activity of bc1 complex S.pombe) like antibody; Chaperone ABC1 activity of bc1 complex homolog antibody; Chaperone ABC1 like antibody; Chaperone activity of bc1 complex like antibody; Chaperone activity of bc1 complex like mitochondrial antibody; Chaperone activity of bc1 complex-like antibody; Chaperone-ABC1-like antibody; Coenzyme Q8 homolog antibody; COQ 8 antibody; COQ10D4 antibody; COQ8 antibody; COQ8; S. cerevisiae; homolog of antibody; MGC4849 antibody; mitochondrial antibody; OTTHUMP00000035734 antibody; OTTHUMP00000035737 antibody; SCAR9 antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Atypical kinase ADCK3, mitochondrial protein (1-220AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

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

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
產品提供形式：

Liquid
應用范圍：

ELISA, IHC

推薦稀釋比：

Application	Recommended Dilution
IHC	1:20-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
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.

產品評價

靶點詳情

功能：

Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration. Its substrate specificity is unclear: does not show any protein kinase activity. Probably acts as a small molecule kinase, possibly a lipid kinase that phosphorylates a prenyl lipid in the ubiquinone biosynthesis pathway, as suggested by its ability to bind coenzyme Q lipid intermediates. Shows an unusual selectivity for binding ADP over ATP.
基因功能參考文獻：
1. Loss of COQ8a results in cerebellar ataxia and coenzyme Q deficiency. PMID: 27499294
2. ADCK3/COQ8 localises to mitochondrial cristae and is targeted to this organelle via the presence of an N-terminal localisation signal PMID: 26866375
3. work reveals Mg(2+)-dependent ATPase activity of ADCK3, providing strong support for the theoretical prediction of this protein being a functional atypical kinase. PMID: 25540914
4. a structural foundation for investigating the role of transmembrane association in regulating the biological activity of ADCK3 PMID: 25216398
5. Mitochondrial ADCK3 employs an atypical protein kinase-like fold to enable coenzyme Q biosynthesis. PMID: 25498144
6. These observations highlight the importance of screening for a potentially treatable cause, CABC1/ADCK3 mutations PMID: 22036850
7. play an important role in mediating p53-inducible apoptosis through the mitochondrial pathway. PMID: 11888884
8. CABC1 gene mutations in four ubiquinone-deficient patients in three distinct families were reported. PMID: 18319072
9. Five additional mutations in ADCK3 were found in three patients with sporadic ataxia, including one known to have CoQ10 deficiency in muscle. PMID: 18319074
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相關疾病：

Coenzyme Q10 deficiency, primary, 4 (COQ10D4)
亞細胞定位：

Mitochondrion. Membrane; Single-pass membrane protein.
蛋白家族：

Protein kinase superfamily, ADCK protein kinase family
組織特異性：

Widely expressed, with highest levels in adrenal gland, heart, pancreas, nasal mucosa, stomach, uterus and skeletal muscle.
數據庫鏈接：

HGNC: 16812

OMIM: 606980

KEGG: hsa:56997

STRING: 9606.ENSP00000355739

UniGene: Hs.118241

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