Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPNCIR147] to FLCN - BSA and Azide free
- Suitable for: WB
- Reacts with: Mouse, Rat, Human
製品名Anti-FLCN antibody [EPNCIR147] - BSA and Azide free
FLCN 一次抗体 製品一覧
製品の詳細Rabbit monoclonal [EPNCIR147] to FLCN - BSA and Azide free
アプリケーション適用あり: WBmore details
適用なし: Flow Cyt,ICC,ICC/IF,IHC-P or IP
種交差性交差種: Mouse, Rat, Human
Recombinant fragment within Human FLCN. The exact sequence is proprietary.
ab248039 is the carrier-free version of ab124885 This format is designed for use in antibody labeling, including fluorochromes, metal isotopes, oligonucleotides, enzymes.
Our carrier-free formats are supplied in a buffer free of BSA, sodium azide and glycerol for higher conjugation efficiency.
Use our conjugation kits for antibody conjugates that are ready-to-use in as little as 20 minutes with <1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.
Ab248039 is compatible with the Maxpar® Antibody Labeling Kit from Fluidigm.
Maxpar® is a trademark of Fluidigm Canada Inc.
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.
保存方法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.
Concentration information loading...
Our Abpromise guarantee covers the use of ab248039 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use at an assay dependent concentration. Detects a band of approximately 70 kDa (predicted molecular weight: 64 kDa).|
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
- 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 data was developed using ab124885, the same antibody clone in a different buffer formulation.
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.