| 產(chǎn)品編號 | bsm-33363M-BF647 |
| 英文名稱1 | Mouse Anti-TTR/Prealbumin/BF647 Conjugated antibody |
| 中文名稱 | BF647標(biāo)記的轉(zhuǎn)甲狀腺素蛋白/前白蛋白單克隆抗體 |
| 別 名 | Transthyretin; 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. |
| 規(guī)格價(jià)格 | 100ul/2980元 購買 大包裝/詢價(jià) |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 生長因子和激素 轉(zhuǎn)運(yùn)蛋白 |
| 抗體來源 | Mouse |
| 克隆類型 | Monoclonal |
| 克 隆 號 | 6B11 |
| 交叉反應(yīng) | Human, |
| 產(chǎn)品應(yīng)用 | ICC=1:50-200 IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 14kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | Recombinant human TTR Protein |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein G |
| 儲 存 液 | Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4. |
| 保存條件 | 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. |
| 產(chǎn)品介紹 |
background: 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: Entrez Gene: 7276 Human Entrez Gene: 22139 Mouse Omim: 176300 Human SwissProt: P27731 Chicken SwissProt: P02766 Human SwissProt: P07309 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 轉(zhuǎn)甲狀腺素(transthyretin，TTR)蛋白由127個(gè)氨基酸組成，在生理?xiàng)l件下4個(gè)TTR蛋白單體分子結(jié)合一個(gè)T4單體分子形成聚合體，存在于血液中參與甲狀腺素的轉(zhuǎn)運(yùn)。TTR蛋白基因發(fā)生遺傳性突變以及在其他因素作用下TTR蛋白聚合體不穩(wěn)定，容易分離形成單體。立體結(jié)構(gòu)發(fā)生變化的TTR單體，進(jìn)一步重合形成蛋白纖維沉積于全身組織、臟器的細(xì)胞間質(zhì)，引起末梢神經(jīng)、自主神經(jīng)感覺障礙以及全身癥狀為特征的綜合臨床癥狀，稱為家族性多發(fā)性神經(jīng)性損害(familial amyloidotic polyneuropathy，F(xiàn)AP)。 |
