货号：07-730

品牌：Merck Millipore

规格：

目录价：￥4264.00

市场价格：￥3624.40

会员价格：￥3411.20

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Neurofibromin (NF-1) Antibody | 07-730 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Upstate (Millipore) View All» Specificity: Neurofibromin (NF-1) View All» Molecular Weight: 250kDa View All» Immunogen: synthetic peptide corresponding to amino acids 2787-2802 (CNSGRTRHGSASQVQK) of human Neurofibromin (NF-1) View All» Isotype: IgG View All» Species Reactivity:

• Human

• Mouse
  View All» Quality Assurance: Routinely evaluated by immunoblot in RIPA lysates from A431 cells View All» Purification Method: Protein A purfied View All» Storage Conditions: 2 years at -20°C from date of shipment View All» UniProt Number: P21359 View All» Entrez Gene Number:
  • NM_000267.2

  • NM_001042492.1
    View All» Gene Symbol:
    • NF1

    • VRNF

    • neurofibromin

    • Neurofibromin

    • DKFZp686J1293

    • WSS

    • NFNS
      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» Entrez Gene Summary: 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. 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» Brand Family: Upstate View All» Product Name: Anti-Neurofibromin (NF-1) Antibody | 07-730 View All» Antibody Type: Polyclonal Antibody View All» Qty/Pk: 200 µg View All» Format: Purified View All» Host: Rabbit View All»
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