merck millipore,默克密理愽
  • merck millipore,默克密理博,04-426,Anti-TSC1 Antibody, rabbit monoclonal

  • merck millipore,默克密理博,04-426,Anti-TSC1 Antibody, rabbit monoclonal

    产品名称：Anti-TSC1 Antibody, rabbit monoclonal
    产品型号：04-426
    Please note that this product will not be available for sale after March 15, 2015. Please select one of the other antibodies against this target. Anti-TSC1 Antibody detects level of TSC1 & has been  More>>

    merck millipore,默克密理博,04-426,Anti-TSC1 Antibody, rabbit monoclonal

  • 产品介绍
  • merck millipore,默克密理博,04-426,Anti-TSC1 Antibody, rabbit monoclonal

    重要规格表

    品种反应性 主要应用 宿主 格式 抗体类型
    Human OnlyWBRbSemi-PurifiedMonoclonal Antibody
    描述
    产品目录编号 04-426
    品牌系列 Upstate
    商名
    • Upstate
    描述 Anti-TSC1 Antibody, rabbit monoclonal
    Alternate Names
    • tuberous sclerosis 1 protein
    • hamartin
    产品信息
    格式 Semi-Purified
    应用
    应用 Please note that this product will not be available for sale after March 15, 2015. Please select one of the other antibodies against this target. Anti-TSC1 Antibody detects level of TSC1 & has been published & validated for use in WB, IH(P).
    主要应用
    • Western Blotting
    生物信息
    免疫原品种 KLH-conjugated synthetic peptide corresponding to the C-terminal region of human TSC1/Hamartin.
    表位 C-terminus
    宿主 Rabbit
    特异性 Recognizes the C-terminal domain of human TSC1.
    同种型 IgG
    品种反应性 Human Only
    抗体类型 Monoclonal Antibody
    Entrez基因编号
    • NM_001008567.1
    • NM_000368.3
    基因符号
    • MGC86987
    • TSC1
    • TSC
    • hamartin
    • LAM
    • KIAA0243
    • Hamartin
    UniProt编号
    • Q92574
    UniProt汇总 FUNCTION: SwissProt: Q92574 # Implicated as a tumor suppressor. May have a function in vesicular transport. Interaction between TSC1 and TSC2 may facilitate vesicular docking.
    SIZE: 1164 amino acids; 129767 Da
    SUBUNIT: Interacts with TSC2. In the absence of TSC2, TSC1 self- aggregates. Interacts with DOCK7.
    SUBCELLULAR LOCATION: Cytoplasm. Membrane; Peripheral membrane protein. Note=At steady state found in association with membranes.
    TISSUE SPECIFICITY: Highly expressed in skeletal muscle, followed by heart, brain, placenta, pancreas, lung, liver and kidney. Also expressed in embryonic kidney cells.
    DOMAIN:SwissProt: Q92574 The C-terminal putative coiled-coil domain is necessary for interaction with TSC2.
    PTM: Phosphorylation at Ser-505 does not affect interaction with TSC2. & Phosphorylated upon DNA damage, probably by ATM or ATR.
    DISEASE: SwissProt: Q92574 # Defects in TSC1 are the cause of tuberous sclerosis complex (TSC) [MIM:191100]. The molecular basis of TSC is a functional impairement of the hamartin-tuberin 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 TSC1 may be a cause of focal cortical dysplasia of Taylor balloon cell type (FCDBC) [MIM:607341]. FCDBC is a subtype of cortical displasias linked to chronic intractable epilepsy. Cortical dysplasias display a broad spectrum of structural changes, which appear to result from changes in proliferation, migration, differentiation, and apoptosis of neuronal precursors and neurons during cortical development.
    SIMILARITY:
    产品使用声明
    质量保证 Routinely evaluated by immunoblot.
    使用声明
    • 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.
    储存和货运信息
    存储条件 2 years at -20°C from date of shipment
    包装信息
    数量 100 µL

    merck millipore,默克密理博,04-426,Anti-TSC1 Antibody, rabbit monoclonal

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