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| 產地 | 進口、國產 |
| 品牌 | 上海莼試 |
| 保存條件 | Store at -20 °C |
| 貨號 | CS9636 |
| 應用范圍 | WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 |
| CAS編號 | |
| 抗體名 | Anti-NF1/Neurofibromin 1 |
| 克隆性 | |
| 靶點 | 詳見說明書 |
| 適應物種 | 詳見說明書 |
| 形態(tài) | 詳見說明書 |
| 宿主 | 詳見說明書 |
| 亞型 | IgG |
| 標識物 | 詳見說明書 |
| 濃度 | 1mg/1ml% |
| 免疫原 | KLH conjugated synthetic peptide derived from human Neurofibromin 1 C-terminus |
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1型神經抗體說明書 英文名稱 Anti-NF1/Neurofibromin 1
中文名稱 1型神經抗體說明書
別 名 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.
產品屬性：
濃 度 1mg/1ml
規(guī) 格 0.1ml/100μg 0.2ml/200μg
抗體來源 Rabbit
克隆類型 polyclonal
交叉反應 Human, Mouse, Rat, Dog, Horse, Rabbit
產品類型 一抗
研究領域 細胞生物 免疫學 染色質和核信號 神經生物學 信號轉導 表觀遺傳學 G蛋白信號
蛋白分子量 predicted molecular weight: 147/319kDa
性 狀 Lyophilized or Liquid
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 0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide
1型神經抗體說明書 產品應用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500
（石蠟切片需做抗原修復）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
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.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
產品介紹 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 : UniProtKB/Swiss-Prot: P21359.2神經纖維素蛋白首先發(fā)現(xiàn)于神經細胞，是一種抑制蛋白，通過調控Ras基因控制異常細胞生長，并且在cAMP信號傳導通路中起.神經Ⅰ型(neurofibromatosis type 1,NF1)是一種由紊亂引起的神經,屬于常染色體顯性遺傳病，其為1/3500，主要表現(xiàn)為咖啡斑、神經、Lisch(虹膜錯構瘤)等。每3，500個新生兒中就有一個是神經纖維細胞瘤I型患者，其臨床表現(xiàn)為表皮或皮下多發(fā)性神經，良性多于惡性，常沿神經干分布。有時，神經會長大，或者發(fā)展到腦和脊髓，大約有一半以上患者。
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