產(chǎn)品編號 | bs-4140R-Gold |
英文名稱 | Rabbit Anti-NF1/Gold Conjugated antibody |
中文名稱 | 膠體金標(biāo)記的1型神經(jīng)纖維瘤抗體 |
別 名 | Neurofibromin 1; DKFZp686J1293; FLJ21220; Neurofibromatosis Noonan syndrome; Neurofibromatosis related protein NF 1; Neurofibromatosis related protein NF1; neurofibromatosis type I; Neurofibromatosis-related protein NF-1; Neurofibromin 1; Neurofibromin truncated; Neurofibromin1; NF 1; NF; NF1; NF1_HUMAN; NFNS; Type 1 Neurofibromatosis; von Recklinghausen disease neurofibromin; von Recklinghausen disease related protein VRNF; VRNF; WATS; Watson disease related protein WSS; Watson syndrome; WSS. |
規(guī)格價格 | 100ul/2980元 購買 大包裝/詢價 |
說 明 書 | 100ul(10nm 15nm 35nm) |
研究領(lǐng)域 | 腫瘤 細(xì)胞生物 免疫學(xué) 染色質(zhì)和核信號 神經(jīng)生物學(xué) 信號轉(zhuǎn)導(dǎo) 表觀遺傳學(xué) G蛋白信號 |
抗體來源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應(yīng) | Human, Mouse, (predicted: Rat, Dog, Horse, Rabbit, ) |
產(chǎn)品應(yīng)用 | IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
分 子 量 | 147/319kDa |
性 狀 | Lyophilized or Liquid |
濃 度 | 0.4mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human Neurofibromin 1 C-terminus |
亞 型 | IgG |
純化方法 | affinity purified by Protein A |
儲 存 液 | 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300. |
保存條件 | Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles. |
產(chǎn)品介紹 |
background: This gene product appears to function as a negative regulator of the ras signal transduction pathway. Mutations in this gene have been linked to neurofibromatosis type 1, juvenile myelomonocytic leukemia and Watson syndrome. The mRNA for this gene is subject to RNA editing (CGA>UGA->Arg1306Term) resulting in premature translation termination. Alternatively spliced transcript variants encoding different isoforms have also been described for this gene. [provided by RefSeq, Jul 2008]. Function: Stimulates the GTPase activity of Ras. NF1 shows greater affinity for Ras GAP, but lower specific activity. May be a regulator of Ras activity. DISEASE: Neurofibromatosis 1 (NF1) [MIM:162200]: A disease characterized by patches of skin pigmentation (cafe-au-lait spots), Lisch nodules of the iris, tumors in the peripheral nervous system and fibromatous skin tumors. Individuals with the disorder have increased susceptibility to the development of benign and malignant tumors. Note=The disease is caused by mutations affecting the gene represented in this entry. Leukemia, juvenile myelomonocytic (JMML) [MIM:607785]: An aggressive pediatric myelodysplastic syndrome/myeloproliferative disorder characterized by malignant transformation in the hematopoietic stem cell compartment with proliferation of differentiated progeny. Patients have splenomegaly, enlarged lymph nodes, rashes, and hemorrhages. Note=The disease is caused by mutations affecting the gene represented in this entry. Watson syndrome (WS) [MIM:193520]: A syndrome characterized by the presence of pulmonary stenosis, cafe-au-lait spots, and mental retardation. It is considered as an atypical form of neurofibromatosis. Note=The disease is caused by mutations affecting the gene represented in this entry. Familial spinal neurofibromatosis (FSNF) [MIM:162210]: Considered to be an alternative form of neurofibromatosis, showing multiple spinal tumors. Note=The disease is caused by mutations affecting the gene represented in this entry. Neurofibromatosis-Noonan syndrome (NFNS) [MIM:601321]: Characterized by manifestations of both NF1 and Noonan syndrome (NS). NS is a disorder characterized by dysmorphic facial features, short stature, hypertelorism, cardiac anomalies, deafness, motor delay, and a bleeding diathesis. Note=The disease is caused by mutations affecting the gene represented in this entry. Colorectal cancer (CRC) [MIM:114500]: A complex disease characterized by malignant lesions arising from the inner wall of the large intestine (the colon) and the rectum. Genetic alterations are often associated with progression from premalignant lesion (adenoma) to invasive adenocarcinoma. Risk factors for cancer of the colon and rectum include colon polyps, long-standing ulcerative colitis, and genetic family history. Note=The gene represented in this entry may be involved in disease pathogenesis. Similarity: Contains 1 CRAL-TRIO domain. Contains 1 Ras-GAP domain. Database links: Entrez Gene: 4763 Human Entrez Gene: 18015 Mouse Omim: 613113 Human SwissProt: P21359 Human SwissProt: Q04690 Mouse Unigene: 113577 Human Unigene: 255596 Mouse Unigene: 10686 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 神經(jīng)纖維素蛋白首先發(fā)現(xiàn)于神經(jīng)細(xì)胞,是一種腫瘤抑制蛋白,通過調(diào)控Ras基因控制異常細(xì)胞生長,并且在cAMP信號傳導(dǎo)通路中起調(diào)節(jié)作用. 神經(jīng)纖維瘤Ⅰ型(neurofibromatosis type 1,NF1)是一種由內(nèi)分泌紊亂引起的神經(jīng)纖維瘤,屬于常染色體顯性遺傳病,其發(fā)病率為1/3500,主要表現(xiàn)為咖啡斑、神經(jīng)纖維瘤、Lisch結(jié)節(jié)(虹膜錯構(gòu)瘤)等。每3,500個新生兒中就有一個是神經(jīng)纖維細(xì)胞瘤I型患者,其臨床表現(xiàn)為表皮或皮下多發(fā)性神經(jīng)纖維瘤,良性多于惡性,常沿神經(jīng)干分布。有時,神經(jīng)纖維瘤會長大,或者發(fā)展到腦和脊髓,大約有一半以上患者智力低下。 |
| 国产精品久久久久久裸体 | 台湾天天成人综合娱乐网 | 三级片国产无遮挡白浆 | 国产91无码精品秘 入口! | 特级婬片A片AAA毛片咕噜咕噜 | 亚洲AV无码久久蜜桃 | 国产精品福利姬在线观看 | 国产一级a一级a免费视频 | 少妇被c 黄 在线视频 | 91又大又爽又黄无码A片 | 陕西少妇性生交BBBBBB | 人妻无码一区二区三区久 | 又大又粗又长又爽的视频 | 亚洲AV人人澡人夜夜人爽人人 | 人人爽人人爽人人爽 | 91人妻人人做人碰人人爽九色-百度 | 国产农村肥熟妇女A片 | 国产又猛又粗又硬又色的视频 | 91在线无码精品秘 入口在线 | 最好看的2019年中文在线观看 | 老妇裸体性激交老太视频 | 香港三日本三级少妇99 | 玩东北老熟女棚户区HD | 在线观看永久免费麻豆 | 美女国产毛片a区内射 | 台湾少妇搡BBBB搡BBBB | 婷婷伊人綜合亞洲綜合網 | 高潮毛片又色又爽免费 | 亚洲天堂无码视频 | 大象传媒色情永久免费版 | 潮喷失禁大喷水aⅴ无码 | 精品人伦一区二区三区蜜桃网站 | 成人午夜电影在线播放 | 无码人妻精品一区二区蜜桃91 | 亚洲精品成人a v无码A片午夜 | 污黄视频在线免费观看 | 污开车视频免费在线观看 | 敌伦交换一区二区三区 | 韩国一区二区三区 | 国产精品扒开腿做爽爽 |