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CT054 Rabbit pAb (bs-4164R)  
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產(chǎn)品編號 bs-4164R
英文名稱 CT054 Rabbit pAb
中文名稱 核黃素轉(zhuǎn)運(yùn)蛋白2抗體
別    名 bA371L19.1; BVVLS; C20orf54; C20orf54provided by HGNC; Chromosome 20 open reading frame 54; hRFT2; MGC10698; S52A3_HUMAN; RFT2; Riboflavin transporter 2.  
Specific References  (1)     |     bs-4164R has been referenced in 1 publications.
[IF=3.454] Anandam KY et al. Effect of the pro-inflammatory cytokine TNF-α on intestinal riboflavin uptake: Inhibition mediated via transcriptional mechanism(s). (2018) American Journal of Physiology-Cell Physiology.  WB ;  Mouse.  
研究領(lǐng)域 腫瘤  免疫學(xué)  信號轉(zhuǎn)導(dǎo)  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse,Rat (predicted: Human,Cow,Dog)
產(chǎn)品應(yīng)用 WB=1:500-2000,Flow-Cyt=3ug/Test
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 51 kDa
檢測分子量
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 CT054 : 391-469/469 
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 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



產(chǎn)品圖片
Sample: Testis (Rat) Lysate at 40 ug Primary: Anti-CT054 (bs-4164R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 51 kD Observed band size: 51 kD
Blank control: Mouse kidney. Primary Antibody (green line): Rabbit Anti-CT054 antibody (bs-4164R) Dilution: 3μg /10^6 cells; Isotype Control Antibody (orange line): Rabbit IgG . Secondary Antibody : Goat anti-rabbit IgG-PE Dilution: 1μg /test. Protocol The cells were incubated in 5%BSA to block non-specific protein-protein interactions for 30 min at at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed.
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