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merck millipore,默克密理博,05-1430,Anti-Neurofibromin Antibody, clone NFn27b
产品名称：Anti-Neurofibromin Antibody, clone NFn27b
产品型号：05-1430
This Anti-Neurofibromin Antibody, clone NFn27b is validated for use in WB for the detection of Neurofibromin.
merck millipore,默克密理博,05-1430,Anti-Neurofibromin Antibody, clone NFn27b
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 H WB M Purified Monoclonal Antibody 描述 产品目录编号 05-1430 Replaces MAB5440 描述 Anti-Neurofibromin Antibody, clone NFn27b Alternate Names - Neurofibromin 1 (neurofibromatosis, von Recklinghausen disease,Watson disease)
- Neurofibromatosis-related protein NF-1
- Neurofibromin (neurofibromatosis, type I)
- type I)
- Neurofibromin 1 (neurofibromatosis
背景信息 NF1 stimulates Ras GTPase activity and may modulate RAS activity. Defects in the NF1 gene are involved in causing several diseases, such as: Juvenile Myelomonocytic Leukemia
(JMML), von Recklionghausen Syndrome, neurofibromatosis-Noonan syndrome (NFNS), Watson Syndrome, Familial Spinal Neurofibromatosis, and may be a contributing cause of Colorectal Cancer ( CRC).
产品信息 格式 Purified 控制 - Huvec cell lysates
演示 Purified immunoglobulin. Liquid in PBS. No preservative. 应用 应用 This Anti-Neurofibromin Antibody, clone NFn27b is validated for use in WB for the detection of Neurofibromin. 主要应用 - Western Blotting
生物信息 免疫原品种 N-terminal peptide from human neurofibromin 表位 N-terminus 克隆 NFn27b 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Mouse 特异性 Reacts with neurofibromin (NF1). Strongly reacts with the expected 220-250 full-length forms of neurofibromin in cell extracts; a Mr» 450 kD form is also detected as well as other lower mass bands, which most likely correspond to complexes, isoforms and degradation products. No high molecular mass immunoreactivity is detected with NF1 knockout mouse extracts. 同种型 IgG1 品种反应性 Human Species Reactivity Note Proven to react with human. Expected to react in mouse and rat based on homology. 抗体类型 Monoclonal Antibody Entrez基因编号 - P21359
基因符号 - WSS
- NF1
- VRNF
- DKFZp686J1293
- NFNS
- Neurofibromin
纯化方法 Protein A purfied UniProt编号 - P21359
UniProt汇总 FUNCTION: SwissProt: P21359 # Stimulates the GTPase activity of Ras. NF1 shows greater affinity for Ras GAP, but lower specific activity. May be a regulator of Ras activity.
SIZE: 2839 amino acids; 319372 Da
DISEASE: SwissProt: P21359 # Defects in NF1 are the cause of type 1 neurofibromatosis (NF1) [MIM:162200]; also called Von Recklinghausen syndrome. NF1 is one of the most frequent autosomal dominant diseases (about 1 in 3000). It exhibits full penetrance by the age of 5 years and high mutation rate with 30 to 50% of NF1 patients representing a new mutation. Among the many clinical features of NF1 are patches of skin pigmentation (cafe-au-lait spots), Lisch nodules of the iris, peripheral nervous system associated tumors and fibromatous skin tumors. & Defects in NF1 are a cause of juvenile myelomonocytic leukemia (JMML) [MIM:607785]. JMML is a pediatric myelodysplastic syndrome that constitutes approximately 30% of childhood cases of myelodysplastic syndrome (MDS) and 2% of leukemia. Germline mutations of NF1 account for the association of JMML with type 1 neurofibromatosis (NF1). & Defects in NF1 are the cause of Watson syndrome (WS) [MIM:193520]. WS is characterized by the presence of pulmonary stenosis, cafe-au-lait spots, and mental retardation. WS is considered as an atypical form of NF1. & Defects in NF1 are a cause of familial spinal neurofibromatosis (spinal NF) [MIM:162210]. Familial spinal NF is considered to be an alternative form of neurofibromatosis, showing multiple spinal tumors. & Defects in NF1 are a cause of neurofibromatosis-Noonan syndrome (NFNS) [MIM:601321]. NFNS is 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. & Defects in NF1 may be a cause of colorectal cancer (CRC) [MIM:114500].
SIMILARITY: SwissProt: P21359 ## Contains 1 CRAL-TRIO domain. & Contains 1 Ras-GAP domain.产品使用声明 质量保证 Evaluated by Western Blot in Huvec Cell lysates.
Western Blot Analysis: 1:500 dilution of this lot detected NEUROFIBROMIN on 10 ug of Huvec lysates.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Maintain at 2-8°C in undiluted aliquots for up to 1 year after date of receipt. 包装信息 数量 100 µg
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