- 当前位置：首页 > >Merck Millipore > 生命科学研究
- 生命科学研究
- 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 R, H, M WB M Purified Monoclonal Antibody 描述 产品目录编号 MAB5438 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Neurofibromin Antibody Alternate Names - NF1
产品信息 格式 Purified 控制 - POSITIVE CONTROL:
Neural ganglia, brain (all blood vessels).
演示 Purified immunoglobulin. Liquid in PBS. 应用 应用 Anti-Neurofibromin Antibody is an antibody against Neurofibromin for use in WB. 主要应用 - Western Blotting
应用说明 Western blot: 1-5 μg/mL using ECL
Immunohistochemistry: works weakly on frozen tissue sections. See MAB5440 for IHC work.
Optimal working dilutions must be determined by end user.生物信息 免疫原品种 N-terminal peptide from human neurofibromin. 表位 (excellent for Western blot) 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; other lower mass bands are also labeled which are most likely correspond to 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 RatHumanMouse 抗体类型 Monoclonal Antibody Entrez基因编号 - NM_001042492.1
- NM_000267.2
Entrez基因汇总 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. 基因符号 - WSS
- NF1
- VRNF
- DKFZp686J1293
- NFNS
- neurofibromin
- Neurofibromin
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 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 6 months after date of receipt. 包装信息 数量 100 µg
上一件merck millipore产品：merck millipore,默克密理博,100105,A23187, Free Acid, Streptomyces chartreusensis - CAS 52665-69-7 - Calbiochem
下一件merck millipore产品：merck millipore,默克密理博,CC1021,Human Integrin αVβ3, Octyl-β-D-glucopyranoside Formulation
相关产品
merck millipore,默克密理博,574777,Anti-Synapsin I Rabbit pAb
merck millipore,默克密理博,09-723,Anti-RNF2 (Ring1b) Antibody
merck millipore,默克密理博,219671,CFTR Inhibitor II, GlyH-101 - CAS 328541-79-3 - Calbiochem
merck millipore,默克密理博,555558,Rho/SRF Pathway Inhibitor, CCG-1423 - CAS 285986-88-1 - Calbiochem
merck millipore,默克密理博,CC1021,Human Integrin αVβ3, Octyl-β-D-glucopyranoside Formulation
merck millipore,默克密理博,14-840,AMPK (α1, β1, γ1) Protein, active, 10 µg
merck millipore,默克密理博,ABC397,Anti-MGAT1 Antibody
merck millipore,默克密理博,07-441,Anti-dimethyl-Histone H3 (Lys9) Antibody
merck millipore,默克密理博,2017-OP,OmniPur A.C.E. Sequencing Water - CAS 7732-18-5 - Calbiochem