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中文名稱：
QRSL1兔多克隆抗體
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貨號：
CSB-PA887948ESR2HU
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規格：
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圖片：
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其他：
產品詳情
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產品名稱：
Rabbit anti-Homo sapiens (Human) QRSL1 Polyclonal antibody
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Uniprot No.：
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基因名：
QRSL1
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別名：
QRSL1 antibody; Glutamyl-tRNA(Gln) amidotransferase subunit A antibody; mitochondrial antibody; Glu-AdT subunit A antibody; EC 6.3.5.7 antibody; Glutaminyl-tRNA synthase-like protein 1 antibody
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宿主：
Rabbit
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反應種屬：
Human
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免疫原：
Recombinant Human Glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial protein (289-528AA)
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免疫原種屬：
Homo sapiens (Human)
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標記方式：
Non-conjugated
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克隆類型：
Polyclonal
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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 0.02% sodium azide, 50% glycerol, pH7.3.
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產品提供形式：
Liquid
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應用范圍：
ELISA, IHC
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推薦稀釋比：
| Application |
Recommended Dilution |
| IHC |
1:20-1:200 |
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Protocols：
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儲存條件：
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.
靶點詳情
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功能：
Allows the formation of correctly charged Gln-tRNA(Gln) through the transamidation of misacylated Glu-tRNA(Gln) in the mitochondria. The reaction takes place in the presence of glutamine and ATP through an activated gamma-phospho-Glu-tRNA(Gln).
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基因功能參考文獻：
- Homozygous intronic variant (c.850-3A > G) in the QRSL1 was identified in a child with cardiomyopathy with early-onset brain disease. PMID: 29440775
- Studies showed in vitro Gln-tRNA(Gln) formation catalyzed by the recombinant mtGluRS and hGatCAB. PMID: 19805282
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相關疾病：
Defects in QRSL1 may play a role in mitochondrial disorders characterized by combined respiratory chain complex deficiencies.
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亞細胞定位：
Mitochondrion.
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蛋白家族：
Amidase family, GatA subfamily
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數據庫鏈接：
Most popular with customers
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