97久久久精品综合88久久_亚洲国产精品一_久热热国产久热_97操操操_北条麻妃在线免费观看_精品国自产拍天天拍

掃碼關(guān)注公眾號(hào)           掃碼咨詢(xún)技術(shù)支持           掃碼咨詢(xún)技術(shù)服務(wù)
  
客服熱線:400-901-9800  客服QQ:4009019800  技術(shù)答疑  技術(shù)支持  質(zhì)量反饋  關(guān)于我們  聯(lián)系我們
亚洲精品动漫,亚洲视频免费一区,黄网站在线播放
Mouse Anti-TTR/Prealbumin/APC Conjugated antibody (bsm-33363M-APC)
訂購(gòu)熱線:400-901-9800
訂購(gòu)郵箱:sales@bioss.com.cn
訂購(gòu)QQ:  400-901-9800
技術(shù)支持:techsupport@bioss.com.cn
說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bsm-33363M-APC
英文名稱(chēng) Mouse Anti-TTR/Prealbumin/APC Conjugated antibody
中文名稱(chēng) APC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(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 生長(zhǎng)因子和激素  轉(zhuǎn)運(yùn)蛋白  
抗體來(lái)源 Mouse
克隆類(lèi)型 Monoclonal
克 隆 號(hào) 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(chǔ) 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.05M PB, pH 7.5.
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

Entrez Gene: 24856 Rat

Omim: 176300 Human

SwissProt: P27731 Chicken

SwissProt: O46375 Cow

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(jué)障礙以及全身癥狀為特征的綜合臨床癥狀,稱(chēng)為家族性多發(fā)性神經(jīng)性損害(familial amyloidotic polyneuropathy,F(xiàn)AP)。
版權(quán)所有 2004-2026 www.kastlife.cn 北京博奧森生物技術(shù)有限公司
通過(guò)國(guó)際質(zhì)量管理體系ISO 9001:2015 GB/T 19001-2016    證書(shū)編號(hào): 00124Q34771R2M/1100
通過(guò)國(guó)際醫(yī)療器械-質(zhì)量管理體系ISO 13485:2016 GB/T 42061-2022    證書(shū)編號(hào): CQC24QY10047R0M/1100
京ICP備05066980號(hào)-1         京公網(wǎng)安備110107000727號(hào)
主站蜘蛛池模板: 日韩精品欧美在线成人 | 久久精品中文字幕一区二区三区 | 少妇又色又爽又高潮极品 | 国产高清不卡一区二区 | 久久五月婷 | 国内精品人妻无码久久久影院蜜桃 | 少妇人妻偷人精品无码视频 | 亚洲精品性 | 亚洲男同帅GAY片在线观看 | 国产福利在线观看视频 | 精品人妻少妇一区二区三区 | 97超碰97 | 亚洲精品久久久久58 | 免费亚洲视频在线观看 | 色久综合一二码 | 两个人看的www免费 国产一区二区三区18 | 7777精品伊人久久久大香线蕉 | 中文字幕色偷偷人妻久久 | 国产中文字幕一区二区 | 亚洲av极品无码专区亚洲av | 毛片一级免费看 | 伊人久久国产 | 中文字幕无码日本欧美大片 | 国产精品一区二区三区久久久久 | 日本激情视频在线播放 | 真实人与人性恔配视频 | 亚洲乱码中文字幕小综合 | 91天仙tv国产福利精品 | 天天久久久 | av中文字幕在线免费观看 | 中文字幕国产视频 | 国产AⅤ爽AV久久久久玉浦团 | 亚洲涩涩av | 日韩国产一级片 | 久草片免费福利资源 | 91视频在线观看网 | 久久99久久99精品免视看看 | JAPANESE人妻中文字幕 | 国产欧美一区二区三区久久人妖 | 国产精品自产拍在线观看 | 国产在线精品国自产拍影院同性 |