Product nameAnti-FLCN antibody [EPNCIR147]
See all FLCN primary antibodies
DescriptionRabbit monoclonal [EPNCIR147] to FLCN
Tested applicationsSuitable for: WBmore details
Unsuitable for: Flow Cyt,ICC,IHC-P or IP
Species reactivityReacts with: Mouse, Rat, Human
Recombinant fragment within Human FLCN. The exact sequence is proprietary.
- 293T and NCCIT cell lysates
This antibody was developed as part of a collaboration between the National Cancer Institute's Center for Cancer Research and the lab of Marston Linehan. View antibodies from NCI Center for Cancer Research Collaboration.
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
Storage instructionsShipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
Storage bufferpH: 7.40
Preservative: 0.05% Sodium azide
Constituents: 40% Glycerol, 9.85% Tris glycine, 50% Tissue culture supernatant
Concentration information loading...
PurityTissue culture supernatant
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.
|WB||1/1000 - 1/10000. Detects a band of approximately 70 kDa (predicted molecular weight: 64 kDa).|
FunctionMay 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 specificityExpressed in most tissues tested, including skin, lung, kidney, heart, testis and stomach.
Involvement in diseaseDefects 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 similaritiesBelongs to the folliculin family.
Developmental stageExpressed in fetal lung, kidney, liver, and brain.
modificationsPhosphorylated. Several different phosphorylated forms exist.
Cellular localizationCytoplasm. Nucleus. Mainly localized in the nucleus. Co-localizes with FNIP1 and FNIP2 in the cytoplasm.
- Information by UniProt
- BHD antibody
- BHD skin lesion fibrofolliculoma protein antibody
- Birt Hogg Dube syndrome protein antibody
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.
All lanes : 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?
This product has been referenced in:
- Li J et al. Myeloid Folliculin balances mTOR activation to maintain innate immunity homeostasis. JCI Insight 5:N/A (2019). Read more (PubMed: 30843872) »
- Kennedy JC et al. Loss of FLCN inhibits canonical WNT signaling via TFE3. Hum Mol Genet 28:3270-3281 (2019). Read more (PubMed: 31272105) »