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产品中心-北京博奥森生物技术有限公司
Recombinant Human TTR Protein, N-His (bs-105721P)  
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20ug/980.00元
50ug/1880.00元
100ug/2680.00元
大包装/询价
产品编号 bs-105721P
英文名称 Recombinant Human TTR Protein, N-His
中文名称
别    名 Transthyretin, Prealbumin, TBPA, TTR, PALB, ATTR  
理论分子量 16.07 kDa
检测分子量
性    状 Lyophilized
纯    度 >90% as determined by SDS-PAGE.
缓 冲 液 Lyophilized from a solution in PBS pH 7.4, 0.02% NLS, 1mM EDTA, 4% Trehalose, 1% Mannitol.
保存条件 Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品介绍 This gene encodes transthyretin, one of the three prealbumins including alpha-1-antitrypsin, transthyretin and orosomucoid. Transthyretin is a carrier protein; it transports thyroid hormones in the plasma and cerebrospinal fluid, and also transports retinol (vitamin A) in the plasma. The protein consists of a tetramer of identical subunits. More than 80 different mutations in this gene have been reported; most mutations are related to amyloid deposition, affecting predominantly peripheral nerve and/or the heart, and a small portion of the gene mutations is non-amyloidogenic. The diseases caused by mutations include amyloidotic polyneuropathy, euthyroid hyperthyroxinaemia, amyloidotic vitreous opacities, cardiomyopathy, oculoleptomeningeal amyloidosis, meningocerebrovascular amyloidosis, carpal tunnel syndrome, etc. [provided by RefSeq]

Gene ID:
7276

转甲状腺素(transthyretin,TTR)蛋白由127个氨基酸组成,在生理条件下4个TTR蛋白单体分子结合一个T4单体分子形成聚合体,存在于血液中参与甲状腺素的转运。TTR蛋白基因发生遗传性突变以及在其他因素作用下TTR蛋白聚合体不稳定,容易分离形成单体。立体结构发生变化的TTR单体,进一步重合形成蛋白纤维沉积于全身组织、脏器的细胞间质,引起末梢神经、自主神经感觉障碍以及全身症状为特征的综合临床症状,称为家族性多发性神经性损害(familial amyloidotic polyneuropathy,
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