Anti-CT054【科瑞奥博】

产品中心

当前位置：首页>产品中心

Anti-CT054

货号： bs-4164R 基本售价： 1380.0 元规格： 100ul

规格：100ul: 价格：1380.00元

规格：200ul: 价格：2200.00元

产品信息

产品编号: bs-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.

规格价格: 100ul/1380元购买 200ul/2200元购买大包装/询价

说明书: 100ul 200ul

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, Rat, Dog, Cow,

产品应用: WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.

分子量: 51kDa

细胞定位: 细胞膜

性状: Lyophilized or Liquid

浓度: 1mg/ml

免疫原: KLH conjugated synthetic peptide derived from human CT054/C20orf54:391-469/469

亚型: IgG

纯化方法: affinity purified by Protein A

储存液: 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

保存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed: PubMed

产品介绍

background:
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: 113278Human

SwissProt: Q9NQ40Human

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

产品图片: 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