產(chǎn)品編號(hào) | bs-41140R |
英文名稱(chēng) | Rabbit Anti-TTR/Prealbumin antibody |
中文名稱(chēng) | 轉(zhuǎn)甲狀腺素蛋白/前白蛋白抗體 |
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. |
研究領(lǐng)域 | 生長(zhǎng)因子和激素 轉(zhuǎn)運(yùn)蛋白 |
抗體來(lái)源 | Rabbit |
克隆類(lèi)型 | Polyclonal |
交叉反應(yīng) | Human,Mouse,Rat |
產(chǎn)品應(yīng)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 | 14 kDa |
檢測(cè)分子量 | |
細(xì)胞定位 | 細(xì)胞漿 分泌型蛋白 |
性 狀 | Liquid |
濃 度 | 1mg/ml |
免 疫 原 | Recombinant human TTR protein |
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(xiàng) | 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 |
Transthyretin (TTR) is a transport protein in the serum and cerebrospinal fluid that carries the thyroid hormone thyroxine (T4) and retinol-binding protein bound to retinol. This is how transthyretin gained its name: transports thyroxine and retinol. The liver secretes transthyretin into the blood, and the choroid plexus secretes TTR into the cerebrospinal fluid. TTR was originally called prealbumin (or thyroxine-binding prealbumin) because it ran faster than albumin on electrophoresis gels. 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. Gene ID: 7276 Database links: Entrez Gene: 7276 Human Entrez Gene: 22139 Mouse Omim: 176300 Human SwissProt: P27731 Chicken SwissProt: P02766 Human SwissProt: P07309 Mouse |
產(chǎn)品圖片 | |
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