FITC标记的线粒体核糖核酸酶P蛋白3抗体
产品名称： FITC标记的线粒体核糖核酸酶P蛋白3抗体
英文名称： Anti-MRPP3/FITC
产品编号： HZ-17807R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-MRPP3/FITC Conjugated antibody
FITC标记的线粒体核糖核酸酶P蛋白3抗体
| 英文名称 | Anti-MRPP3/FITC |
| 中文名称 | FITC标记的线粒体核糖核酸酶P蛋白3抗体 |
| 别 名 | Kiaa0391; KIAA0391 gene; Mitochondrial ribonuclease P protein 3; Mitochondrial RNase P protein 3; Mitochondrial RNase P subunit 3; MRRP3_HUMAN; PRORP; Proteinaceous RNase P. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | |
| 抗体来源 | |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 62kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human MRPP3 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | background: Chromosome 14 contains about 700 genes and 106 million base pairs and makes up about 3.5% of human cellular DNA. Chromosome 14 encodes the presinilin 1 (PSEN1) gene, which is one of the three key genes associated with the development of Alzheimer's disease. The SERPINA1 gene is located on chromosome 14 and when defective leads to the genetic disorder ?-antitrypsin deficiency. This disorder is characterized by severe lung complications and liver dysfunction. Notably, the immunoglobulin heavy chain locus is found on chromosome 14 and has been identified as a fusion with the chromosome 19 encoded protein BCL3 in the (14;19) translocations found in a variety of B cell malignancies. The KIAA0391 gene product has been provisionally designated KIAA0391 pending further characterization. Function: Functions in mitochondrial tRNA maturation. Part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/RG9MTD1, MRPP2/HSD17B10 and MRPP3/KIAA0391, which cleaves tRNA molecules in their 5'-ends. Subunit: Interacts with MRPP1/TRMT10C and MRPP2/HSD17B10. Subcellular Location: Mitochondrion. Database links: Entrez Gene: 9692 Human Omim: 609947 Human SwissProt: O15091 Human Unigene: 458487 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
第14染色体含有约700个基因和1亿600万个碱基对，约占人类细胞DNA的3.5%。染色体14编码前列宁1（PSEN1）基因，它是与阿尔茨海默病的发展相关的三个关键基因之一。SerpIA1基因位于14号染色体上，当有缺陷时会导致遗传障碍？抗胰蛋白酶缺乏症。这种疾病的特点是严重的肺部并发症和肝功能不全。值得注意的是，免疫球蛋白重链基因座发现于染色体14上，并且已经被鉴定为在多种B细胞恶性肿瘤中发现的(14;19)易位中与染色体19编码蛋白BCL3融合。KIAA039基因产物已暂时指定KIAA039 1，有待进一步鉴定。