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RabbitAnti-CT054 Rabbit pAb  antibody (bs-21658R)
~~~促销,代码KXJ230206~~~
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-21658R
英文名称 CT054 Rabbit pAb
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  
研究领域 肿瘤  免疫学  信号转导  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, 
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 51 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 CT054 : 211-310/469 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% 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 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

Gene ID:
113278

Database links:

Entrez Gene: 113278 Human

SwissProt: Q9NQ40 Human



产品图片
Sample: A431(Human) Cell Lysate at 30 ug Uterus (Mouse) Lysate at 40 ug Primary: Anti-CT054 (bs-21658R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 51 kD Observed band size: 51 kD
Sample: A431(Human) Cell Lysate at 30 ug Primary: Anti-CT054 (bs-21658R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 51 kD Observed band size: 51 kD
Sample: A431(Human) Cell Lysate at 30 ug Uterus (Mouse) Lysate at 40 ug Lung (Mouse) Lysate at 40 ug Hela(Human) Cell Lysate at 30 ug Primary: Anti- CT054 (bs-21658R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 51 kD Observed band size: 51 kD
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