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05-1430 | Anti-Neurofibromin Antibody, clone NFn27b

100 µg  
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      Key Specifications Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H WB M Purified Monoclonal Antibody
      Catalogue Number05-1430
      DescriptionAnti-Neurofibromin Antibody, clone NFn27b
      Alternate Names
      • Neurofibromin
      • Neurofibromatosis-related protein NF-1
      Background InformationNF1 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).
      Product Information
      • Huvec cell lysates
      PresentationPurified immunoglobulin. Liquid in PBS. No preservative.
      ApplicationThis Anti-Neurofibromin Antibody, clone NFn27b is validated for use in WB for the detection of Neurofibromin.
      Key Applications
      • Western Blotting
      Biological Information
      ImmunogenN-terminal peptide from human neurofibromin
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      SpecificityReacts 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.
      Species Reactivity
      • Human
      Species Reactivity NoteProven to react with human. Expected to react in mouse and rat based on homology.
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Gene Symbol
      • NF1
      • VRNF
      • DKFZp686J1293
      • NFNS
      • Neurofibromin
      • WSS
      Purification MethodProtein A Purfied
      UniProt Number
      UniProt SummaryFUNCTION: 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.
      Molecular WeightApprox. 320 kDa.
      Physicochemical Information
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blot in Huvec Cell lysates.
      Western Blot Analysis: 1:500 dilution of this lot detected NEUROFIBROMIN on 10 ug of Huvec lysates.
      Usage Statement
      • 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.
      Storage and Shipping Information
      Storage ConditionsMaintain at 2-8°C in undiluted aliquots for up to 1 year after date of receipt.
      Packaging Information
      Material Size100 µg
      Transport Information
      Supplemental Information

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      Life Science Research > Antibodies and Assays > Primary Antibodies