Anti-FLCN抗体[EPNCIR147] (ab124885)



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.

应用 Ab评论 说明
WB 1/1000 - 1/10000. Detects a band of approximately 70 kDa (predicted molecular weight: 64 kDa).
  • 应用说明Is unsuitable for Flow Cyt,ICC,IHC-P or IP.
  • 靶标

    • 功能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.
    • 组织特异性Expressed in most tissues tested, including skin, lung, kidney, heart, testis and stomach.
    • 疾病相关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.
    • 序列相似性Belongs to the folliculin family.
    • 发展阶段Expressed in fetal lung, kidney, liver, and brain.
    • 翻译后修饰Phosphorylated. Several different phosphorylated forms exist.
    • 细胞定位Cytoplasm. Nucleus. Mainly localized in the nucleus. Co-localizes with FNIP1 and FNIP2 in the cytoplasm.
    • Information by UniProt
    • 数据库链接
    • 别名
      • 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] 图像

    • 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.

      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)参考文献

    ab124885 has not yet been referenced specifically in any publications.

    Product Wall

    There are currently no Abreviews or Questions for ab124885.
    Please use the links above to contact us or submit feedback about this product.