Overview

  • Product name

  • Description

    Rabbit polyclonal to FLCN
  • Host species

    Rabbit
  • Tested applications

    Suitable for: ICC/IF, IHC-Pmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Chimpanzee, Gorilla
  • Immunogen

    Synthetic peptide within Human FLCN aa 1-50 (N terminal). The exact sequence is proprietary. NP_659434.2 .
    Sequence:

    MNAIVALCHFCELHGPRTLFCTEVLHAPLPQGDGNEDSPGQGEQAEEEEG


    Database link: Q8NFG4

  • Positive control

    • Human linitis plastica stomach cancer and Human ovarian carcinoma tissues.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
  • Storage buffer

    Preservative: 0.09% Sodium azide
    Constituents: 0.1% BSA, 99% Tris buffered saline
  • Concentration information loading...
  • Purity

    Immunogen affinity purified
  • Purification notes

    ab176707 was affinity purified using an epitope specific to FLCN immobilized on solid support.
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab176707 in the following tested applications.

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

Application Abreviews Notes
ICC/IF 1/50 - 1/500.
IHC-P 1/100 - 1/500. Epitope exposure is recommended. Epitope exposure with citrate buffer will enhance staining. Likely to work with frozen sections.

Target

  • Function

    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.
  • Tissue specificity

    Expressed in most tissues tested, including skin, lung, kidney, heart, testis and stomach.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the folliculin family.
  • Developmental stage

    Expressed in fetal lung, kidney, liver, and brain.
  • Post-translational
    modifications

    Phosphorylated. Several different phosphorylated forms exist.
  • Cellular localization

    Cytoplasm. Nucleus. Mainly localized in the nucleus. Co-localizes with FNIP1 and FNIP2 in the cytoplasm.
  • Information by UniProt
  • Database links

  • Alternative names

    • 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

Images

  • Immunohistochemical analysis of paraffin embedded Human linitis plastica stomach cancer tissue labeling FLCN with ab176707 at 1/250 dilution, followed by DAB staining.

  • Immunohistochemical analysis of paraffin embedded Human ovarian carcinoma tissue labeling FLCN with ab176707 at 1/250 dilution, followed by DAB staining.

References

ab176707 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

Application
Western blot
Sample
Human Cell lysate - whole cell (BHD kidney tumor cell lines: UOK257 (FLCN-) & UOK2)
Gel Running Conditions
Reduced Denaturing (Bio-Rad 8-16% gradient Tris-Glycine gel)
Loading amount
25 µg
Specification
BHD kidney tumor cell lines: UOK257 (FLCN-) & UOK2
Blocking step
BSA as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: RT°C

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Verified customer

Submitted Apr 01 2016

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