货号：05-1430

品牌：Merck Millipore

规格：100MIG

目录价：￥3552.00

市场价格：￥3019.20

会员价格：￥2841.60

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Neurofibromin Antibody, clone NFn27b | 05-1430 View All» Replaces: MAB5440 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Specificity: 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. View All» Molecular Weight: Approx. 320 kDa. View All» Epitope: N-terminus View All» Immunogen: N-terminal peptide from human neurofibromin View All» Clone: NFn27b View All» Isotype: IgG1 View All» Background Information: 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). View All» Species Reactivity: Human View All» Species Reactivity Note: Proven to react with human. Expected to react in mouse and rat based on homology. View All» Control: Huvec cell lysates View All» Quality Assurance: 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. View All» Purification Method: Protein A purfied View All» Presentation: Purified immunoglobulin. Liquid in PBS. No preservative. View All» Storage Conditions: Maintain at 2-8°C in undiluted aliquots for up to 1 year after date of receipt. View All» UniProt Number: P21359 View All» Entrez Gene Number: P21359 View All» Gene Symbol:

• NF1

• VRNF

• DKFZp686J1293

• NFNS

• Neurofibromin

• WSS
  View All» Alternate Names:
  • Neurofibromatosis-related protein NF-1

  • Neurofibromin (neurofibromatosis, type I)

  • Neurofibromin 1 (neurofibromatosis, von Recklinghausen disease,Watson disease)
    View All» 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. View All» Key Applications: Western Blotting View All» UniProt Summary: 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. View All» Product Name: Anti- Neurofibromin, clone NFn27b View All» Concentration: 1 mg/mL View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 μg View All» Format: Purified View All» Host: Mouse View All»
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