| 產(chǎn)品編號(hào) | bs-13209R |
| 英文名稱 | FOXRED1 Rabbit pAb |
| 中文名稱 | 單跨膜蛋白FOXRED1抗體 |
| 別 名 | FAD dependent oxidoreductase domain containing 1; FAD dependent oxidoreductase domain containing protein 1; FAD-dependent oxidoreductase domain-containing protein 1; FOXRED 1; FOXRED1; FP634; FXRD1_HUMAN; H17. |
| 研究領(lǐng)域 | 腫瘤 細(xì)胞生物 神經(jīng)生物學(xué) 信號(hào)轉(zhuǎn)導(dǎo) 新陳代謝 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Mouse (predicted: Human,Rat,Rabbit,Pig,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 | 54 kDa |
| 檢測(cè)分子量 | |
| 細(xì)胞定位 | 細(xì)胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human FOXRED1: 251-350/486 |
| 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 |
FOXRED1 is a 486 amino acid single-pass membrane protein. Utilizing FAD as a cofactor, FOXRED1 may act as a chaperone protein essential for the function of mitochondrial complex I. Mutations to FOXRED1 may result in mitochondrial complex I deficiency (MT-C1D), which results in a wide range of clinical maladies from lethal neonatal disease to adult onset neurodegenerative disorders. Common phenotypes of MT-C1D include cardiomyopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. FOXRED1 exists as three alternatively spliced isoforms and is encoded by a gene mapping to human chromosome 11q24.2. With approximately 135 million base pairs and 1,400 genes, chromosome 11 makes up around 4% of human genomic DNA and is considered a gene and disease association dense chromosome. Subcellular Location: Membrane; Single-pass membrane protein (Potential). DISEASE: Defects in FOXRED1 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. SWISS: Q96CU9 Gene ID: 55572 Database links: Entrez Gene: 55572 Human Entrez Gene: 235169 Mouse GenBank: NP_060017.1 Human Omim: 613622 Human SwissProt: Q4R510 Cynomolgus Monkey SwissProt: Q96CU9 Human SwissProt: Q3TQB2 Mouse Unigene: 317190 Human Unigene: 138512 Mouse Leigh綜合征的發(fā)生率占新生兒的1/40,000.具有不同的基因類型,但臨床具有共性特點(diǎn),一般發(fā)病在1歲或以后,表現(xiàn)為肌張力減退,發(fā)作性嘔吐,共濟(jì)失調(diào),舞蹈徐動(dòng)癥和過度通氣,腦病表現(xiàn)為喪失語言發(fā)育能力,運(yùn)動(dòng)異常表現(xiàn)為痙攣性運(yùn)動(dòng)和異常呼吸節(jié)律,出現(xiàn)腦干或基底節(jié)損害體征和聽力喪失,小腦損害導(dǎo)致共濟(jì)失調(diào),眼震和張力失常.眼科癥狀表現(xiàn)為視力喪失和眼肌麻痹.出現(xiàn)亞臨床的周圍神經(jīng)病,出現(xiàn)神經(jīng)傳導(dǎo)速度減慢45%.臨床體征可以在感染或糖尿病后出現(xiàn).病程進(jìn)展出現(xiàn)運(yùn)動(dòng)或智能減退.常在發(fā)病后2年內(nèi)死亡. |
| 產(chǎn)品圖片 |
Sample: Lung (Mouse) Lysate at 40 ug
Primary: Anti-FOXRED1 (bs-13209R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 54 kD
Observed band size: 50 kD
Sample: Cerebellum (Mouse) Lysate at 40 ug
Primary: Anti-FOXRED1 (bs-13209R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 54 kD
Observed band size: 54 kD
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