| 產(chǎn)品編號 | bs-19505R |
| 英文名稱 | NUBPL Rabbit pAb |
| 中文名稱 | 核苷酸結(jié)合蛋白樣NUBPL抗體 |
| 別 名 | C14orf127; FLJ12660; huInd1; IND1 homolog; Iron-sulfur protein NUBPL; Nubpl; NUBPL_HUMAN; nucleotide binding protein like; Nucleotide-binding protein-like. |
| 研究領(lǐng)域 | 細(xì)胞生物 表觀遺傳學(xué) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 克 隆 號 | |
| 交叉反應(yīng) | Human (predicted: Mouse,Rat,Sheep,Cow,Dog,Horse) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 34 kDa |
| 檢測分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human NUBPL: 1-100/319 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
This gene encodes a member of the Mrp/NBP35 ATP-binding proteins family. The encoded protein is required for the assembly of the respiratory chain NADH dehydrogenase (complex I), an oligomeric enzymatic complex located in the inner mitochondrial membrane. Mutations in this gene cause mitochondrial complex I deficiency. Alternative splicing results in multiple transcript variants. [provided by RefSeq, May 2014] Function: Required for the assembly of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I). May deliver of one or more Fe-S clusters to complex I subunits. Subcellular Location: Mitochondrion. Tissue Specificity: Highest expression in liver and kidney. expressed at significant levels in small intestine and brain (at protein level). DISEASE: Defects in NUBPL 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 manifestations 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. Similarity: Belongs to the Mrp/NBP35 ATP-binding proteins family. SWISS: Q8TB37 Gene ID: 80224 Database links: Entrez Gene: 80224 Human Entrez Gene: 76826 Mouse Omim: 613621 Human SwissProt: Q8TB37 Human SwissProt: Q9CWD8 Mouse Unigene: 288981 Human Unigene: 244781 Mouse Unigene: 13455 Rat |
| 產(chǎn)品圖片 |
Sample:
Hela(Human) Cell Lysate at 30 ug
Primary: Anti-NUBPL (bs-19505R) at 1/500 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 34 kD
Observed band size: 34 kD
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