QRSL1 Antibody
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货号:CSB-PA887948ESR2HU
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规格:¥440
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促销:
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图片:
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其他:
产品详情
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产品名称:Rabbit anti-Homo sapiens (Human) QRSL1 Polyclonal antibody
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Uniprot No.:Q9H0R6
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基因名:QRSL1
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别名: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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反应种属:Human
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免疫原:Recombinant Human Glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial protein (289-528AA)
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免疫原种属:Homo sapiens (Human)
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标记方式:Non-conjugated
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克隆类型:Polyclonal
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抗体亚型:IgG
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纯化方式:Antigen Affinity Purified
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浓度:It differs from different batches. Please contact us to confirm it.
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保存缓冲液:PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
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产品提供形式:Liquid
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应用范围:ELISA, IHC
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推荐稀释比:
Application Recommended Dilution IHC 1:20-1:200 -
Protocols:
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储存条件:Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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货期: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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靶点详情
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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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基因功能参考文献:
- 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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相关疾病:Defects in QRSL1 may play a role in mitochondrial disorders characterized by combined respiratory chain complex deficiencies.
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亚细胞定位:Mitochondrion.
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蛋白家族:Amidase family, GatA subfamily
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数据库链接:
HGNC: 21020
KEGG: hsa:55278
STRING: 9606.ENSP00000358042
UniGene: Hs.406917
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