货号：MAB3792

品牌：Merck Millipore

规格：EA

目录价：询价

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-TSC2 Antibody, clone 3G1.2 | MAB3792 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: Recognizes human TSC2. This antibody detects a band of approximately 180 kDa in Western blot analysis of HEK293 cell lysate. View All» Immunogen: Synthetic peptide corresponding to amino acid residues 1441-1460 of human TSC2 (Accession # P49815). Sequence is 100% identical in chimpanzee, mouse, and rat. View All» Clone: 3G1.2 View All» Isotype: IgG2b View All» Background Information: Tuberous sclerosis (TSC) is a potentially devastating disease associated with mutations in tumor suppressor genes TSC1 and TSC2 that function as a complex to suppress signaling in the mTOR/S6K/4E-BP pathway. View All» Species Reactivity:

• Human

• Rat
  View All» Application Notes: Western blot: 2 μg/mL Optimal working dilutions must be determined by the end user. View All» Control: Western blot positive control: HEK293 cell line View All» Presentation: Protein A purified immunoglobulin. Liquid in 0.02M Phosphate Buffer, 0.25M NaCl, with 0.1% sodium azide. View All» Storage Conditions: Maintain at 2-8°C for up to 12 months from date of receipt. View All» UniProt Number: P49815 View All» Entrez Gene Number:
  • NM_021055.1

  • NM_021056.1

  • NM_000548.3

  • NM_001077183.1
    View All» Gene Symbol:
    • TSC2

    • TSC4

    • tuberin

    • LAM

    • FLJ43106

    • Tuberin
      View All» Alternate Names: Tuberin 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: Mutations in this gene lead to tuberous sclerosis complex. Its gene product is believed to be a tumor suppressor and is able to stimulate specific GTPases. The protein associates with hamartin in a cytosolic complex, possibly acting as a chaperone for hamartin. Alternative splicing results in multiple transcript variants encoding different isoforms. View All» UniProt Summary: FUNCTION: SwissProt: P49815 # Implicated as a tumor suppressor. May have a function in vesicular transport, but may also play a role in the regulation of cell growth arrest and in the regulation of transcription mediated by steroid receptors. Interaction between TSC1 and TSC2 may facilitate vesicular docking. Specifically stimulates the intrinsic GTPase activity of the Ras-related protein RAP1A and RAB5. Suggesting a possible mechanism for its role in regulating cellular growth. Mutations in TSC2 leads to constitutive activation of RAP1A in tumors.SIZE: 1807 amino acids; 200749 Da SUBUNIT: Interacts with TSC1. May also interact with the adapter molecule RABEP1. The final complex contains TSC2 and RABEP1 linked to RAB5 (Probable). Interacts with HSPA1 and HSPA8.SUBCELLULAR LOCATION: Cytoplasm. Membrane; Peripheral membrane protein. Note=At steady state found in association with membranes.TISSUE SPECIFICITY: Liver, brain, heart, lymphocytes, fibroblasts, biliary epithelium, pancreas, skeletal muscle, kidney, lung and placenta.PTM: Phosphorylation at Ser-1387, Ser-1418 or Ser-1420 does not affect interaction with TSC1.DISEASE: SwissProt: P49815 # Defects in TSC2 are the cause of tuberous sclerosis complex (TSC) [MIM:191100]. The molecular basis of TSC is a functional impairment of the tuberin-hamartin complex. TSC is an autosomal dominant multi-system disorder that affects especially the brain, kidneys, heart, and skin. TSC is characterized by hamartomas (benign overgrowths predominantly of a cell or tissue type that occurs normally in the organ) and hamartias (developmental abnormalities of tissue combination). Clinical symptoms can range from benign hypopigmented macules of the skin to profound mental retardation with intractable seizures to premature death from a variety of disease-associated causes. & Defects in TSC2 are a cause of lymphangioleiomyomatosis (LAM) [MIM:606690]. LAM is a progressive and often fatal lung disease characterized by a diffuse proliferation of abnormal smooth muscle cells in the lungs. It affects almost exclusively young women and can occur as an isolated disorder or in association with tuberous sclerosis complex.SIMILARITY: SwissProt: P49815 ## Contains 1 Rap-GAP domain. View All» Brand Family: Chemicon View All» Product Name: Anti-TSC2 Antibody, clone 3G1.2 | MAB3792 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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