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★转甲状腺素蛋白抗体说明书 分子生物学：质粒抽提、PCR、Q-PCR、RT-PCR、分子生物学：基因合成、引物合成、基因测序、载体构建等

★蛋白工程：原核、哺乳动物蛋白表达系统等

★病毒包装：腺病毒、慢病毒等

★抗体工程：磁珠分选、病理染色、WB、ELISA、IP、IF、IHC、FACS、Confocal等等

★细胞工程：细胞表型分析(凋亡、增殖、周期、迁移、侵袭、修复、克隆形成)、细胞培养、细胞膜制备、稳定细胞株构建、细胞RNAi技术等等。

实验流程：

转甲状腺素蛋白抗体说明书 英文名称  Anti-Transthyretin/Prealbumin

中文名称  转甲状腺素蛋白抗体说明书 

别    名 Amyloid polyneuropathy; Amyloidosis I; ATTR; Dysprealbuminemic euthyroidal hyperthyroxinemia; Dystransthyretinemic hyperthyroxinemia; HsT2651; PALB; Prealbumin amyloidosis type I; Senile systemic amyloidosis; TBPA; Transthyretin; TTR; TTR protein; prealbumin; TTHY_HUMAN.

产品属性：

浓    度  1mg/1ml

规 格  0.1ml/100μg 0.2ml/200μg

抗体来源  Rabbit

克隆类型   polyclonal

交叉反应   Human, Mouse, Rat

产品类型   一抗  

研究领域    生长因子和激素 转运蛋白

蛋白分子量  predicted molecular weight: 14kDa 

性    状   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human Transthyretin

亚    型  IgG

纯化方法   affinity purified by Protein A

储 存 液   0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide

转甲状腺素蛋白抗体说明书 产品应用   WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 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.  

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. 

Important Note  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]

Function : Thyroid hormone-binding protein. Probably transports thyroxine from the bloodstream to the brain.

Subunit : Homotetramer. Dimer of dimers. In the homotetramer, subunits assemble around a central channel that can accommodate two ligand molecules. Interacts with RBP4.

Subcellular Location : Secreted. Cytoplasm.

Tissue Specificity : Detected in serum and cerebrospinal fluid (at protein level). Highly expressed in choroid plexus epithelial cells. Detected in retina pigment epithelium and liver.

Post-translational modifications : Not glycosylated under normal conditions. Following unfolding, caused for example by variant AMYL-TTR 'Gly-38', the cryptic Asn-118 site is exposed and glycosylated by STT3B-containing OST complex, leading to its degradation by the ER-associated degradation (ERAD) pathway.

DISEASE : Defects in TTR are the cause of amyloidosis transthyretin-related (AMYL-TTR) [MIM:105210]. A hereditary eneralized amyloidosis due to transthyretin amyloid deposition. Protein fibrils can form in different tissues leading to amyloid polyneuropathies, amyloidotic cardiomyopathy, carpal tunnel syndrome, systemic senile amyloidosis. The disease includes leptomeningeal amyloidosis that is characterized by primary involvement of the central nervous system. Neuropathologic examination shows amyloid in the walls of leptomeningeal vessels, in pia arachnoid, and subpial deposits. Some patients also develop vitreous amyloid deposition that leads to visual impairment (oculoleptomeningeal amyloidosis). Clinical features include seizures, stroke-like episodes, dementia, psychomotor deterioration, variable amyloid deposition in the vitreous humor.

Defects in TTR are a cause of hyperthyroxinemia dystransthyretinemic euthyroidal (HTDE) [MIM:145680]. It is a condition characterized by elevation of total and free thyroxine in healthy, euthyroid persons without detectable binding protein abnormalities.

Defects in TTR are a cause of carpal tunnel syndrome type 1 (CTS1) [MIM:115430]. It is a condition characterized by entrapment of the median nerve within the carpal tunnel. Symptoms include burning pain and paresthesias involving the ventral surface of the hand and fingers which may radiate proximally. Impairment of sensation in the distribution of the median nerve and thenar muscle atrophy may occur. This condition may be associated with repetitive occupational trauma, wrist injuries, amyloid neuropathies, rheumatoid arthritis.

Similarity : Belongs to the transthyretin family.

Database links :

UniProtKB/Swiss-Prot: P02766.1

Entrez Gene: 7276 Human

Entrez Gene: 22139 Mouse

Entrez Gene: 24856 Rat

Omim: 176300 Human

SwissProt: P27731 Chicken

SwissProt: O46375 Cow

SwissProt: P02766 Human

SwissProt: P07309 Mouse

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、自主神经以及全身为特征的综合临床，称为家族性多发性神经性损害(familial amyloidotic polyneuropathy，FAP)。

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