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一般說明
Neurofibromin (UniProt: P21359; also known as Neurofibromatosis-related protein NF-1) is encoded by the NF1 gene (Gene ID: 4763) in human. Neurofibromin is a GTPase-activating protein that negatively regulatres RAS/MAOK pathway by enhancing the hydrolysis of Ras-bound GTP. Hence, it can display tumor suppressor properties. Neurofibromin has been detected in brain, peripheral nerves, lung, colon, and muscle tissues. It has a CRAL-TRIO domain (aa 1580-1738) that facilitates its binding to phospholipids. It binds primarily glycerophospholipids with monounsaturated C18:1 and/or C16:1 fatty acid moieties and a phosphatidylethanolamine or phosphatidylcholine head group. Neurofibromin also has a Ras-GTP domain (aa 1235-1451). Mutations in NF1 gene are known to cause Neurofibromatosis type I that involved tumors in the peripheral nervous system and fibromatous skin tumors. Some mutations are also known to cause colorectal cancer and juvenile leukemia. Six different isoforms of Neurofibromin have been described that are produced by alternative splicing. (Ref.: Rossi, S., et al. (2018). Mod. Pathol. 31(1), 160-168).
特異性
Clone NFC specifically detects human Neurofibromin C. it targets an epitope with in the C-terminal region.
免疫原
Epitope: C-terminus
Recombinant fragment corresponding to 281 amino acids from the C-terminal region of human Neurofibromin C.
應用
Anti-Neurofibromin C, clone NFC, Cat. No. MABE1820, is a highly specific mouse monoclonal antibody that targets Neurofibromin and has been tested for use in Immunohistochemistry (Paraffin).
Immunohistochemistry (Paraffin) Analysis: A representative lot detected Neurofibromin C in Immunohistochemistry applications (Rossi, S., et. al. (2018). Mod Pathol. 31(1):160-168).
Western Blotting Analysis: A representative lot detected Neurofibromin C lysate from human NF1 +/+ cells, but not in -/- cells or NF1+/+ cells transfected with siRNA targeting neurofibromin (Reuss, DE.,et al., (2014). Acta Neuropathol. 127(4); 565-572).
Western Blotting Analysis: A representative lot detected Neurofibromin C lysate from human NF1 +/+ cells, but not in -/- cells or NF1+/+ cells transfected with siRNA targeting neurofibromin (Reuss, DE.,et al., (2014). Acta Neuropathol. 127(4); 565-572).
Research Category
Epigenetics & Nuclear Function
Epigenetics & Nuclear Function
品質
Evaluated by Immunohistochemistry (Paraffin) in human kidney tissue sections.
Immunohistochemistry (Paraffin) Analysis: A 1:50 dilution of this antibody detected Neurofibromin C in human kidney tissue sections.
Immunohistochemistry (Paraffin) Analysis: A 1:50 dilution of this antibody detected Neurofibromin C in human kidney tissue sections.
標靶描述
319.37 kDa calculated.
外觀
Protein G purified
Format: Purified
Purified mouse monoclonal antibody IgG1 in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
儲存和穩定性
Stable for 1 year at 2-8°C from date of receipt.
其他說明
Concentration: Please refer to lot specific datasheet.
免責聲明
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.
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Sabrina Rossi et al.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 31(1), 160-168 (2017-09-02)
An increasing body of evidence supports the involvement of NF1 mutations, constitutional or somatic, in the pathogenesis of gastrointestinal stromal tumors (GISTs). Due to the large size of the NF1 locus, the existence of multiple pseudogenes and the wide spectrum
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