製品の概要

アプリケーション

Our Abpromise guarantee covers the use of ab124885 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

アプリケーション Abreviews 特記事項
WB 1/1000 - 1/10000. Detects a band of approximately 70 kDa (predicted molecular weight: 64 kDa).
  • 追加情報Is unsuitable for Flow Cyt,ICC,IHC-P or IP.
  • ターゲット情報

    • 機能May play a role in the pathogenesis of an uncommon form of kidney cancer through its association with an inherited disorder of the hair follicle (fibrofolliculomas). May be a tumor suppressor. May be involved in colorectal tumorigenesis. May be involved in energy and/or nutrient sensing through the AMPK and mTOR signaling pathways. May regulate phosphorylation of RPS6KB1.
    • 組織特異性Expressed in most tissues tested, including skin, lung, kidney, heart, testis and stomach.
    • 関連疾患Defects in FLCN are the cause of Birt-Hogg-Dube syndrome (BHD) [MIM:135150]. BHD is a rare autosomal dominant genodermatosis characterized by hair follicle hamartomas (fibrofolliculomas), kidney tumors, and spontaneous pneumothorax. Fibrofolliculomas are part of the triad of BHD skin lesions that also includes trichodiscomas and acrochordons. Onset of this dermatologic condition is invariably in adulthood. BHD is associated with a variety of histologic types of renal tumors, including chromophobe renal cell carcinoma (RCC), benign renal oncocytoma, clear-cell RCC and papillary type I RCC. Multiple lipomas, angiolipomas, and parathyroid adenomas are also seen in patients affected with this disease. The majority of mutations are predicted to prematurely terminate the protein.
      Defects in FLCN are in some cases a cause of primary spontaneous pneumothorax (PSP) [MIM:173600]. PSP is a condition in which air is present in the pleural space in the absence of a precipitating event, such as trauma or lung disease. This results in secondary collapse of the lung, either partially or completely, and some degree of hypoxia. PSP is relatively common, with an incidence between 7.4-18/100'000 for men and 1.2-6/100'000 for women and a dose-dependent, increased risk among smokers. Most cases are sporadic, typically occurring in tall, thin men aged 10-30 years and generally while at rest. Familial PSP is rarer and usually is inherited as an autosomal dominant condition with reduced penetrance, although X-linked recessive and autosomal recessive inheritance have also been suggested.
      Note=Defects in FLCN may be involved in renal cell carcinoma.
    • 配列類似性Belongs to the folliculin family.
    • 発生段階Expressed in fetal lung, kidney, liver, and brain.
    • 翻訳後修飾Phosphorylated. Several different phosphorylated forms exist.
    • 細胞内局在Cytoplasm. Nucleus. Mainly localized in the nucleus. Co-localizes with FNIP1 and FNIP2 in the cytoplasm.
    • Information by UniProt
    • 参照データベース
    • 別名
      • BHD antibody
      • BHD skin lesion fibrofolliculoma protein antibody
      • Birt Hogg Dube syndrome protein antibody
      • Birt-Hogg-Dube syndrome protein antibody
      • DKFZp547A118 antibody
      • FLCL antibody
      • Flcn antibody
      • FLCN_HUMAN antibody
      • FLJ45004 antibody
      • FLJ99377 antibody
      • Folliculin antibody
      • MGC17998 antibody
      • MGC23445 antibody
      see all

    Anti-FLCN antibody [EPNCIR147] 画像

    • All lanes : Anti-FLCN antibody [EPNCIR147] (ab124885) at 1/1000 dilution

      Lane 1 : 293T cell lysate
      Lane 2 : NCCIT cell lysate

      Lysates/proteins at 10 µg per lane.

      Secondary
      HRP labelled goat anti-rabbit at 1/2000 dilution

      Predicted band size : 64 kDa
      Observed band size : 70 kDa (why is the actual band size different from the predicted?)

    Anti-FLCN antibody [EPNCIR147] (ab124885) 使用論文

    ab124885 has not yet been referenced specifically in any publications.

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