| 产品编号 | bs-13209R |
| 英文名称 | FOXRED1 Rabbit pAb |
| 中文名称 | 单跨膜蛋白FOXRED1抗体 |
| 别 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. |
| 研究领域 | 肿瘤 细胞生物 神经生物学 信号转导 新陈代谢 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Mouse, (predicted: Human, Rat, Dog, Pig, Horse, Rabbit, ) |
| 产品应用 | WB=1:500-2000 ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理论分子量 | 54 kDa |
| 检测分子量 | |
| 细胞定位 | 细胞膜 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human FOXRED1: 251-350/486 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 缓 冲 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 产品介绍 |
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综合征的发生率占新生儿的1/40,000.具有不同的基因类型,但临床具有共性特点,一般发病在1岁或以后,表现为肌张力减退,发作性呕吐,共济失调,舞蹈徐动症和过度通气,脑病表现为丧失语言发育能力,运动异常表现为痉挛性运动和异常呼吸节律,出现脑干或基底节损害体征和听力丧失,小脑损害导致共济失调,眼震和张力失常.眼科症状表现为视力丧失和眼肌麻痹.出现亚临床的周围神经病,出现神经传导速度减慢45%.临床体征可以在感染或糖尿病后出现.病程进展出现运动或智能减退.常在发病后2年内死亡. |
| 产品图片 |
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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| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
