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    CT054 Rabbit pAb (bs-21658R)  
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    產(chǎn)品編號 bs-21658R
    英文名稱(chēng) CT054 Rabbit pAb
    中文名稱(chēng) 核黃素轉運蛋白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  
    研究領(lǐng)域 腫瘤  免疫學(xué)  信號轉導  新陳代謝  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    克 隆 號
    交叉反應 Human,Mouse
    產(chǎn)品應用 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 51 kDa
    檢測分子量
    細胞定位 細胞膜 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human CT054 : 211-310/469 
    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 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: 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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