FITC标记的NDUF3蛋白抗体
产品名称： FITC标记的NDUF3蛋白抗体
英文名称： Anti-NDUF3/FITC
产品编号： HZ-19067R-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-NDUF3/FITC Conjugated antibody
FITC标记的NDUF3蛋白抗体
| 英文名称 | Anti-NDUF3/FITC |
| 中文名称 | FITC标记的NDUF3蛋白抗体 |
| 别 名 | C3orf60; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3; NDUF3_HUMAN; ndufaf3. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 细胞生物 神经生物学 信号转导 转录调节因子 新陈代谢 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Pig, Horse, Rabbit, |
| 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 | 20kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human NDUF3 |
| 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: This gene encodes a mitochondrial complex I assembly protein that interacts with complex I subunits. Mutations in this gene cause mitochondrial complex I deficiency, a fatal neonatal disorder of the oxidative phosphorylation system. Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Jul 2009] Function: Essential factor for the assembly of mitochondrial NADH:ubiquinone oxidoreductase complex (complex I). Subunit: Interacts with NDUFAF4, NDUFS2 and NDUFS3. Subcellular Location: Nucleus. Mitochondrion inner membrane. DISEASE: Defects in NDUFAF3 are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. Database links: Entrez Gene: 25915 Human Entrez Gene: 56769 Rat Omim: 612911 Human SwissProt: Q9BU61 Human SwissProt: O08776 Rat Unigene: 31387 Human Unigene: 40118 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
该基因编码线粒体复合物I组装蛋白，与复杂的I亚基相互作用。该基因的突变导致线粒体复合体I缺乏，一种致命的新生儿氧化磷酸化系统紊乱。另一种编码不同的异构体的剪接转录变体已被鉴定。[ RefSeq，JUL 2009 ]