ADCK3 Antibody
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中文名稱：ADCK3兔多克隆抗體
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貨號(hào)：CSB-PA004383GA01HU
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規(guī)格：￥3,900
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其他：
產(chǎn)品詳情
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Uniprot No.：
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基因名：COQ8A
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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
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宿主：Rabbit
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反應(yīng)種屬：Human,Mouse,Rat
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免疫原：Human CABC1
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免疫原種屬：Homo sapiens (Human)
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抗體亞型：IgG
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純化方式：Antigen Affinity purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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產(chǎn)品提供形式：Liquid
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應(yīng)用范圍：ELISA,WB,IF
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Protocols：
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儲(chǔ)存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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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.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產(chǎn)品評(píng)價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情
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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.
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基因功能參考文獻(xiàn)：
- Loss of COQ8a results in cerebellar ataxia and coenzyme Q deficiency. PMID: 27499294
- ADCK3/COQ8 localises to mitochondrial cristae and is targeted to this organelle via the presence of an N-terminal localisation signal PMID: 26866375
- 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
- a structural foundation for investigating the role of transmembrane association in regulating the biological activity of ADCK3 PMID: 25216398
- Mitochondrial ADCK3 employs an atypical protein kinase-like fold to enable coenzyme Q biosynthesis. PMID: 25498144
- These observations highlight the importance of screening for a potentially treatable cause, CABC1/ADCK3 mutations PMID: 22036850
- play an important role in mediating p53-inducible apoptosis through the mitochondrial pathway. PMID: 11888884
- CABC1 gene mutations in four ubiquinone-deficient patients in three distinct families were reported. PMID: 18319072
- 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(guān)疾病：Coenzyme Q10 deficiency, primary, 4 (COQ10D4)
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亞細(xì)胞定位：Mitochondrion. Membrane; Single-pass membrane protein.
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蛋白家族：Protein kinase superfamily, ADCK protein kinase family
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組織特異性：Widely expressed, with highest levels in adrenal gland, heart, pancreas, nasal mucosa, stomach, uterus and skeletal muscle.
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數(shù)據(jù)庫(kù)鏈接：
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