產(chǎn)品編號 | bs-11788R-AP |
英文名稱 | Rabbit Anti-Prion protein PrP/CD230/AP Conjugated antibody |
中文名稱 | 堿性磷酸酶(AP)標(biāo)記的朊病毒蛋白CD230抗體 |
別 名 | AltPrP; ASCR; atal familial insomnia; CD230; CD230 antigen; CJD; Creutzfeld Jakob disease; Gerstmann-Strausler-Scheinker syndrome; GSS; KURU; Major prion protein; PRIO_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 | 細(xì)胞生物 神經(jīng)生物學(xué) 干細(xì)胞 細(xì)菌及病毒 細(xì)胞表面分子 |
抗體來源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應(yīng) | (predicted: Human, Mouse, Rat, Cow, Horse, ) |
產(chǎn)品應(yīng)用 | WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
分 子 量 | 25kDa |
性 狀 | Lyophilized or Liquid |
濃 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human Prion protein PrP/CD230 |
亞 型 | IgG |
純化方法 | affinity purified by Protein A |
儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
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: The function of PrP is still under debate. May play a role in neuronal development and synaptic plasticity. May be required for neuronal myelin sheath maintenance. May play a role in iron uptake and iron homeostasis (By similarity). Isoform 2 may act as a growth suppressor by arresting the cell cycle at the G0/G1 phase. Soluble oligomers are toxic to cultured neuroblastoma cells and induce apoptosis (in vitro). Function: The function of PrP is still under debate. May play a role in neuronal development and synaptic plasticity. May be required for neuronal myelin sheath maintenance. May play a role in iron uptake and iron homeostasis (By similarity). Isoform 2 may act as a growth suppressor by arresting the cell cycle at the G0/G1 phase. Soluble oligomers are toxic to cultured neuroblastoma cells and induce apoptosis (in vitro). Subunit: Monomer and homodimer. Has a tendency to aggregate into amyloid fibrils containing a cross-beta spine, formed by a steric zipper of superposed beta-strands. Soluble oligomers may represent an intermediate stage on the path to fibril formation. Copper binding may promote oligomerization. Interacts with GRB2, APP, ERI3/PRNPIP and SYN1. Mislocalized cytosolically exposed PrP interacts with MGRN1; this interaction alters MGRN1 subcellular location and causes lysosomal enlargement (By similarity). Interacts with KIAA1191. Subcellular Location: Cell membrane. Golgi apparatus and Cytoplasm. Nucleus. Accumulates outside the secretory route in the cytoplasm, from where it relocates to the nucleus. Post-translational modifications: The glycosylation pattern (the amount of mono-, di- and non-glycosylated forms or glycoforms) seems to differ in normal and CJD prion. Isoform 2 is sumoylated by SUMO1. DISEASE: Note=PrP is found in high quantity in the brain of humans and animals infected with neurodegenerative diseases known as transmissible spongiform encephalopathies or prion diseases, like: Creutzfeldt-Jakob disease (CJD), fatal familial insomnia (FFI), Gerstmann-Straussler disease (GSD), Huntington disease-like type 1 (HDL1) and kuru in humans; scrapie in sheep and goat; bovine spongiform encephalopathy (BSE) in cattle; transmissible mink encephalopathy (TME); chronic wasting disease (CWD) of mule deer and elk; feline spongiform encephalopathy (FSE) in cats and exotic ungulate encephalopathy (EUE) in nyala and greater kudu. The prion diseases illustrate three manifestations of CNS degeneration: (1) infectious (2) sporadic and (3) dominantly inherited forms. TME, CWD, BSE, FSE, EUE are all thought to occur after consumption of prion-infected foodstuffs. Defects in PRNP are the cause of Creutzfeldt-Jakob disease (CJD) [MIM:123400]. CJD occurs primarily as a sporadic disorder (1 per million), while 10-15% are familial. Accidental transmission of CJD to humans appears to be iatrogenic (contaminated human growth hormone (HGH), corneal transplantation, electroencephalographic electrode implantation, etc.). Epidemiologic studies have failed to implicate the ingestion of infected annimal meat in the pathogenesis of CJD in human. The triad of microscopic features that characterize the prion diseases consists of (1) spongiform degeneration of neurons, (2) severe astrocytic gliosis that often appears to be out of proportion to the degree of nerve cell loss, and (3) amyloid plaque formation. CJD is characterized by progressive dementia and myoclonic seizures, affecting adults in mid-life. Some patients present sleep disorders, abnormalities of high cortical function, cerebellar and corticospinal disturbances. The disease ends in death after a 3-12 months illness. Defects in PRNP are the cause of fatal familial insomnia (FFI) [MIM:600072]. FFI is an autosomal dominant disorder and is characterized by neuronal degeneration limited to selected thalamic nuclei and progressive insomnia. Defects in PRNP are the cause of Gerstmann-Straussler disease (GSD) [MIM:137440]. GSD is a heterogeneous disorder and was defined as a spinocerebellar ataxia with dementia and plaquelike deposits. GSD incidence is less than 2 per 100 million live births. Defects in PRNP are the cause of Huntington disease-like type 1 (HDL1) [MIM:603218]. HDL1 is an autosomal dominant, early onset neurodegenerative disorder with prominent psychiatric features. Defects in PRNP are the cause of kuru (KURU) [MIM:245300]. Kuru is transmitted during ritualistic cannibalism, among natives of the New Guinea highlands. Patients exhibit various movement disorders like cerebellar abnormalities, rigidity of the limbs, and clonus. Emotional lability is present, and dementia is conspicuously absent. Death usually occurs from 3 to 12 month after onset. Defects in PRNP are the cause of spongiform encephalopathy with neuropsychiatric features (SENF) [MIM:606688]; an autosomal dominant presenile dementia with a rapidly progressive and protracted clinical course. The dementia was characterized clinically by frontotemporal features, including early personality changes. Some patients had memory loss, several showed aggressiveness, hyperorality and verbal stereotypy, others had parkinsonian symptoms. Prion diseases, or transmissible spongiform encephalopathies (TSEs), are manifested as genetic, infectious or sporadic, lethal neurodegenerative disorders involving alterations of the prion protein (PrP). Characteristic of prion diseases, cellular PrP (PrPc) is converted to the disease form, PrPSc, through alterations in the protein folding conformations. PrPc is constitutively expressed in normal adult brain and is sensitive to proteinase K digestion, while the altered PrPSc conformation is resistant to proteases, resulting in a distinct molecular mass after PK treatment. Consistent with the transient infection process of prion diseases, incubation of PrPc with PrPSc both in vitro and in vivo produces PrPc that is resistant to protease degradation. Infectious PrPSc is found at high levels in the brains of animals affected by TSEs, including scrapie in sheep, BSE in cattle and Cruetzfeldt-Jakob disease in humans. Similarity: Belongs to the prion family. Database links: Entrez Gene: 5621 Human Entrez Gene: 19122 Mouse Entrez Gene: 493887 Sheep Omim: 176640 Human SwissProt: P04156 Human SwissProt: P04925 Mouse SwissProt: P23907 Sheep Unigene: 472010 Human Unigene: 610285 Human Unigene: 727471 Human Unigene: 648 Mouse Unigene: 3936 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 亚洲色无色A片一区二区 | 一区二区三区精品 | 99re视频在线观看 | 色欲AV性色av浪潮AV壹牛网 | A级成人婬片免费看 | 亚洲AV无码乱码 | 上海熟妇搡BBBB搡BBBB | 国产aaaa一级毛片 | 免费一看一级毛片少妇丰满2 | 国产精品国产三级国产 | 国精黄黄久久久免 | 国产精品伦人视频免费看三丽医院 | 成人做爰黄 片免费观看 | 亚洲美女一区二区三区 | 91人妻人人爽人人添夜夜爽直播 | 6080午夜精品无码 | 黄色无码在线免费播放 | 国产无 码免费观看少萝 | 亚洲精品国产成人综合久久久久久久久 | 中文字幕在线免费 | 国产精品久久久久久久久动漫 | 日本AⅤ无码乱码国产成人网站 | 国产成人精品 水 国产成人精品AA毛片 | 兴奋视频网站在线观看 | 欧美日韩r级视频手机在线 一区二区三区四区福利视频 | 白丝女疯狂 喷水自慰爽 | 国产A级婬片A片免费妖精 | 午夜大尺度色无码专区 | 国产av成人精品www | 高清无码熟妇在线播放 | 精品秘 无码一区二区久久 无码免费婬AV片在线观看 | 大又大又粗又硬又爽少妇毛片 | 国产91清纯白嫩初高中 | 17c在线精品无码秘 动漫 | 免费观看国产又大又长又粗又黄的A√片 | 欧美精品videosex极品 | 天天操天天日舔舔舔 | 少妇人妻上班偷人露脸 | 亚洲av无码乱码a片秀色直播 | 少妇把腿扒开让我添69式mv |