上海细胞库
人源细胞系| 稳转细胞系| 基因敲除株| 基因点突变细胞株| 基因过表达细胞株| 重组细胞系| 猪的细胞系| 马细胞系| 兔的细胞系| 犬的细胞系| 山羊的细胞系| 鱼的细胞系| 猴的细胞系| 仓鼠的细胞系| 狗的细胞系| 牛的细胞| 大鼠细胞系| 小鼠细胞系| 其他细胞系|
人源细胞系| 稳转细胞系| 基因敲除株| 基因点突变细胞株| 基因过表达细胞株| 重组细胞系| 猪的细胞系| 马细胞系| 兔的细胞系| 犬的细胞系| 山羊的细胞系| 鱼的细胞系| 猴的细胞系| 仓鼠的细胞系| 狗的细胞系| 牛的细胞| 大鼠细胞系| 小鼠细胞系| 其他细胞系|
| 规格 | 价格 | 库存 |
|---|---|---|
| 50ul | ￥ 1200 | 9 |
| 100ul | ￥ 1900 | 5 |
| 200ul | ￥ 2900 | 3 |
| 产品编号 | Ys-4164R |
| 英文名称 | CT054 |
| 中文名称 | 核黄素转运蛋白2抗体 |
| 别 名 | bA371L19.1; BVVLS; C20orf54; C20orf54provided by HGNC; Chromosome 20 open reading frame 54; hRFT2; MGC10698; S52A3_HUMAN; RFT2; Riboflavin transporter 2. |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Mouse, Rat, (predicted: Human, Dog, Cow, ) |
| 产品应用 | WB=1:500-2000 ELISA=1:5000-10000 Flow-Cyt=3ug/Test not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理论分子量 | 51kDa |
| 细胞定位 | 细胞膜 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human CT054 : 391-469/469 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 缓 冲 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store at -20 °C 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 | CT054 is a Riboflavin transporter. Riboflavin transport is Na+-independent but moderately pH-sensitive. Activity is strongly inhibited by riboflavin analogs, such as lumiflavin, flavin mononucleotide (FMN) and flavin adenine dinucleotide (FAD), and to a lesser extent by amiloride. Function: Riboflavin transporter. Riboflavin transport is Na(+)-independent but moderately pH-sensitive. Activity is strongly inhibited by riboflavin analogs, such as lumiflavin, flavin mononucleotide (FMN) and flavin adenine dinucleotide (FAD), and to a lesser extent by amiloride. Subcellular Location: Cell membrane; Multi-pass membrane protein. Tissue Specificity: Predominantly expressed in testis. Highly expressed in small intestine and prostate. DISEASE: Defects in RFT2 are the cause of Brown-Vialetto-Van Laere syndrome (BVVLS) [MIM:211530]. BVVLS is rare autosomal recessive neurologic disorder characterized by sensorineural hearing loss and a variety of cranial nerve palsies, which develop over a relatively short period of time in a previously healthy individual. Sensorineural hearing loss may precede the neurological signs. The course is invariably progressive, but the rate of decline is variable within and between families. With disease evolution, long tract signs, lower motor neuron signs, cerebellar ataxia, and lower cranial nerve (III-VI) palsies develop, giving rise to a complex picture resembling amyotrophic lateral sclerosis. Diaphragmatic weakness and respiratory compromise are some of the most distressing features, leading to recurrent chest infections and respiratory failure, which are often the cause of patients' demise. Defects in RFT2 are the cause of Fazio-Londe disease (FALOND) [MIM:211500]. A rare neurological disease characterized by progressive weakness of the muscles innervated by cranial nerves of the lower brain stem. It may present in childhood with severe neurological deterioration with hypotonia, respiratory insufficiency leading to premature death, or later in life with bulbar weakness which progresses to involve motor neurons throughout the neuroaxis. Clinical manifestations include dysarthria, dysphagia, facial weakness, tongue weakness, and fasciculations of the tongue and facial muscles. Similarity: Belongs to the riboflavin transporter family. SWISS: Q9NQ40 |
